A 28-year-old woman was admitted six months before her death because of abdominal pain, anemia and hepatosplenomegaly. Clinical diagnosis by biopsy examination was primary hepatic angiosarcoma. The autopsy revealed about 700 ml of bloody ascites. The spleen weighed 510 g, and was completely replaced by dark grayish-red tumors. The enlarged liver was 6,560 g and revealed multiple tumor nodules. Microscopically, the tumor cells formed irregular vascular spaces. Factor VIII-related antigens were stained in the tumor cells. Zonula adherens was observed also by electron microscopic observation.