Hypohidrotic ectodermal dysplasia (HED) is a rare geneticcondition. It is of recessive autosomic character and linked tothe X chromosome, encompassing a heterogeneous group ofdisorders involving ectoderm-derived tissues. Males are affected by the disease and females play the role of carriers. This disease presents a typical triad: hypohidrosis, oligodontia and hypotrichosis. This article examines the clinical case of a patient who exhibited characteristic signs of HED: dry skin, scarce hair eyebrows and eyelashes, wrinkles and peri-oral and peri-orbital hyperpigmentation, prominent lips and nasal bridge and decreased lachrymal secretion. Intra-oral examination revealed multiple tooth absences, presenceof only eight teeth in the upper jaw and two teeth in the lowerjaw, all with shape alterations and presence of diastemata. Early recognition and dental treatment of HED carrier individuals referred to oral alterations is of the utmost importance, not only to achieve favorable esthetics and functionality but also to allow the decrease facial growth alterations in a short time.