Epithelioid hemangioendothelioma of liver is a very rare tumor of vascular origin, the most peculiar feature of which is that it is composed of endothelial cells closely resembling epithelial cells. We present a primary epithelioid hemangioendothelioma of liver in a 40-year-old male. This tumor was composed of an ill-defined yellowish white, 7 x 4cm sized, firm, solid mass and small satellite nodules in the right lobe of liver. Microscopically, two types of tumor cells-dendritic and epithelioid-were identified. The neoplastic cells infiltrated into sinusoids and intrahepatic veins. The background of tumor showed marked sclerotic change and focal proliferation of bile ductules. Confirmation of the endothelial origin of these cells was provided by positive immunoperoxidase staining for factor VIII-related antigen, and by electron mi-croscopic demonstration of Weibel-Palade body. This is the first case of epithelioid heman- gioendothelioma of liver documented in Korea. We report this case in view of its scarcity and distinctive morphologic features that allow differentiation from sclerosing carcinoma and angiosarcoma.