Harlequin ichthyosis is defined as a genetic disorder of autosomal recessive inheritance, which severely affects the ABCA12 gene. Which is responsible for the coding of an essential protein, for the maintenance of the skin's lipid barrier. However, this pathology is diagnosed with the naked eye due to its patchy appearance of the skin, due to the multiple hyperkeratosis plaques that present fissures (Almendraa & Aranibar, 2017). We must know that the association of other malformations is very frequent, such as; ectropion (eversion of the eyelids), eclabium (everted lips), and hypoplasia of nasal bones, pinnae, and fingers and toes. Therefore, harlequin ichthyosis is the most severe and aggressive form of congenital ichthyosis, which has a low prevalence of approximately 1 / 300,000 births with variable clinical expression, an unfavorable evolution and a reserved prognosis (WHO / PAHO, 2017). Prenatal diagnosis is possible in some ichthyosis and should be evaluated when there is a family history with a genetic pattern that makes transmission possible. The most commonly used techniques may include amniocentesis, villus biopsy, fetal skin biopsy, blood and urine analytical studies (Ávila, Montoya, & Bustillo, 2016). The initial management of these patients includes good and correct hygiene, humid heat in an incubator, high fluid intake to avoid dehydration due to transepidermal losses, and emulsifying ointments. Finally, our patient was also administered empirical antibiotic therapy and morphine intravenously in infusion, prior to transfer to the neonatal intensive care unit, the Nursing Care Process was drawn up in a 4-day neonate with Harlequin Ichthyosis with the objective to restore their state of health through physical assessment and functional patterns. Using the Marjory Gordon model to prioritize nursing interventions and develop a specific care plan based on the needs required by the patient.Harlequin ichthyosis is defined as a genetic disorder of autosomal recessive inheritance, which severely affects the ABCA12 gene. Which is responsible for the coding of an essential protein, for the maintenance of the skin's lipid barrier. However, this pathology is diagnosed with the naked eye due to its patchy appearance of the skin, due to the multiple hyperkeratosis plaques that present fissures (Almendraa & Aranibar, 2017). We must know that the association of other malformations is very frequent, such as; ectropion (eversion of the eyelids), eclabium (everted lips), and hypoplasia of nasal bones, pinnae, and fingers and toes. Therefore, harlequin ichthyosis is the most severe and aggressive form of congenital ichthyosis, which has a low prevalence of approximately 1 / 300,000 births with variable clinical expression, an unfavorable evolution and a reserved prognosis (WHO / PAHO, 2017). Prenatal diagnosis is possible in some ichthyosis and should be evaluated when there is a family history with a genetic pattern that makes transmission possible. The most commonly used techniques may include amniocentesis, villus biopsy, fetal skin biopsy, blood and urine analytical studies (Ávila, Montoya, & Bustillo, 2016). The initial management of these patients includes good and correct hygiene, humid heat in an incubator, high fluid intake to avoid dehydration due to transepidermal losses, and emulsifying ointments. Finally, our patient was also administered empirical antibiotic therapy and morphine intravenously in infusion, prior to transfer to the neonatal intensive care unit, the Nursing Care Process was drawn up in a 4-day neonate with Harlequin Ichthyosis with the objective to restore their state of health through physical assessment and functional patterns. Using the Marjory Gordon model to prioritize nursing interventions and develop a specific care plan based on the needs required by the patient.La ictiosis arlequín se define como un desorden genético de herencia autosómica recesiva, que afecta de manera severa al gen ABCA12. El cual es responsable de la codificación de una proteína esencial, para el mantenimiento de la barrera lipídica cutánea. Sin embargo, esta patología se diagnostica a simple vista por su aspecto parcheado de la piel, debido a las múltiples placas de hiperqueratosis que presentan fisura (Almendraa & Aranibar, 2018). Debemos saber que es muy frecuente la asociación de otras malformaciones, como por ejemplo; ectropión (eversión de los párpados), eclabium (labios evertidos) e hipoplasia de huesos nasales, pabellones auriculares y dedos de manos y pies. Por aquello, la ictiosis arlequín es la forma más grave y agresiva de las ictiosis congénitas, que se presentan una baja prevalencia de un aproximado 1/300.000 nacimientos con expresividad clínica variable, una evolución desfavorable y pronóstico reservad (OMS/OPS, 2017). El diagnóstico prenatal es posible en algunas ictiosis y debe ser evaluado cuando existen antecedentes familiares con un patrón genético que haga posible la transmisión. Las técnicas más emplear pueden incluir amniocentesis, biopsia de vellosidades, biopsia de piel fetal, estudios analíticos de sangre y orina (Ávila, Montoya, & Bustillo, 2016). El manejo inicial de estos pacientes incluye una buena y correcta higiene, calor húmedo en incubadora, alto aporte de líquidos para evitar la deshidratación por las pérdidas transepidérmicas y pomadas emulsificantes. Por último, a nuestro paciente, también se administró antibioterapia empírica y morfina por vía intravenosa en perfusión, previo traslado a la unidad de cuidados intensivos neonatal se procedió a la elaboración del Proceso de Atención de Enfermería en neonato de 4 dias con Ictiosis Arlequín con el objetivo restaurar su estado de salud a través de la valoración física y por patrones funcionales. Utilizando el modelo Marjory Gordon para priorizar las intervenciones en enfermería y elaborar un plan de cuidados específicos en base a las necesidades requeridas por el paciente.