Familial erythrophagocytic lymphohistiocytosis with neonatal presentation

: A neonatal case of familial erythrophagocytic lymphohistiocytosis is presented. Evolution was rapidly fatal under a course in which fever, hepatosplenomegaly, pancytopenia, severe coagulation alteration and jaundice were especially significant. Pathology findings after nine days of life, were all conclusive. Diagnostic difficulties of this uncommon entity in its' congenital form only once previously reported, are emphasized.