Two cases of histiocytic medullary reticulosis occurring in an 18 years old male and a 14 years old female were presented with discussion of their clinicopathologic characteristics and brief literature review, and the latter was verified with autopsy. Although the cutaneous lesions were much more prominent in our cases, the clinical symptoms and signs, rapid fatal course, and hematological features were basically identical to those of previously documented histiocytic medullary reticulosis. On autopsy, the lesions were found to be systemic and showed diffuse proliferation of the atypical hitiocytes engaged in active phagocytosis. Liver, spleen, Iymph nodes, and bone marrow were mainly involved, but the other tissues, such as skin, lungs, brain, kidneys, thyroid, adrenal capsule, pericardium, and thoracic wall, were considerably affected as well.