A case of histiocytic medullary reticulosis in 14-years-old girl was presented, who had characteristic clinical and histiopathologic findings. The patient manifested intermitten fever, general weakness, weight loss, multiple skin lesion and pancytopenia with terminal massive bleeding. The clinical finding with fatal course and hematologic features were basically identical to those of previously documented histiocytic medullary reticulosis and verified with autopsy. On autopsy, systemic proliferation of the actively phagocytic atypical histiocytes was found. The authors report the case with review of pertinent literature.