A case report of histiocytosis X in a 2 years old in­faut, primarily admitted a for jaundice is discussed. Major clinical manifestations included: Jaundice, bi­lateral otitis media. pallor and loss of weight. Clinical examination revealed hepatosplenomegaly. lymphadenopathies and sever anemia. Seborrr.eic eruptions and ptechias appeared over the scalp and other parts of body surface a few days after admission. Blod tests have nol led to final diagnosis. Bone marrow examination follewed by liver and skin biopsies disclosed histiocytosis X. Therapeutic regimen consisting of corticosteroid and vinblastine brought a remarkable clinical remission for the patim.