A 36 years man was admitted for two months, history of fever, weight loss, fatigue and recently gradually increasing jaundice. Physical examination revealed subfebril fever, icter, hepatosplenomegaly and ascites. Laboratory findings showed pancytopenia, hypoproteinemia associated with elevated levels of serum bilirubin, transaminases, alkaline phosphates, glutamyl transpeptidase, lactic dehydrogenase, cholesterol, triglyceride, C-reactive protein, ferritin, and prolonged protrombin time. Bone marrow aspiration and biopsy examination demonstrated too many macrophages displaying erythrophagocytosis. Liver biopsy demonstrated disseminated necrosis areas. The ethiology of the case diagnosed as hemophagocytic syndrome by hlstopathological findings, could not be determined. Clinical picture of the patient deteriorated and he died on day 38 of admission, although he was treated with immunosupressive and pulse steroid therapy. Hemophagocytic syndrome has a clinical picture of fatal course. It should be suspected of an underlying malignant disease, if an infectious process has not been established as an etiologic reason. The treatment is controversial and is directed to the reason, generally.