Self-healing reticulohistiocytosis is a perinatal disease characterized by rapidly developing and involuting benign histiocytic infiltration of the skin and other organ. We had recently experienced a case of a self-healing reticulohistiocytosis in 99-day-old infant who presented with multiple erythematous nodular lesions on face and trunk and pancytopenia. Our case differed from those previously reported in that extracutaneous involvement was found. The patient had pancytopenia, hypertriglyceridemia, hypofibrinogenemia hyperferritinemia and hepatosplenomegaly as well as skin lesions. Bone marrow showed a marked lymphocytosis with many histiocytes. We described a detailed clinical features of this case and reviewed the literatures.