A case of nonlipoid reticuloendotheliosis (Letterer-Siwe's disease) with autopsy is reported. The relationship to essential xanthomatosis (Hand-Schüller-Christian) and to eosinophilic granuloma is discussed. Unusual features of our case, classical in other respects, were the severe leukopenia and thrombocytopenia, the presence of severe edema and ascites, associated with hypoproteinemia.