El síndrome hemofagocítico (SHF) es un trastorno caracterizado por la activación y proliferación no maligna del sistema inmune. Puede ser primario o secundario. La forma primaria está ligada a factores genéticos autosómicos recesivos y la secundaria se presenta subsiguiente a otras enfermedades. La leishmaniasis es la parasitosis que más comúnmente lo gatilla y tiene la característica que resuelve con su tratamiento específico, sin el cual la mortalidad alcanza el 100 %. Se presenta el caso de un niño de 7 meses, oriundo de Paraguay, que ingresa a nuestro Hospital por fiebre y hepatoesplenomegalia, que luego de arribar al diagnóstico y realizar tratamiento específico, evolucionó favorablemente.Hemophagocytic syndrome (HPS) is a disorder characterized by non-malignant activation and proliferation of the immune system. It can be primary or secondary. The primary form is linked to autosomal recessive genetic factors and the secondary form occurs subsequent to other diseases. Leishmaniasis is a parasitic disease with a wide worldwide distribution caused by different species of Leishmania. It is the protozoan that most commonly triggers it and it resolves with its specific treatment. Without it, the mortality rate is 100 %. We report the case of a 7-month-old boy, born in Paraguay, who was admitted at our Hospital with fever and hepatosplenomegaly, who after reaching the diagnosis, received specific treatment and developed a favorable clinical outcome.