Hepatic epithelioid hemangioendothelioma (HEH) is a rare vascular tumor of the liver of unknown etiology. Although HEH is usually characterized by a low grade malignancy and a good long-term prognosis, its growth can be progressive and lead to hepatic failure, extrahepatic metastasis and death. Several different antineoplastic agents have been proposed for cases of nonresectable HEH. We report the case of a 45-year-old man with HEH successfully treated with intravenous infusion of Adriamycin.