Malignant epithelioid hemangioendothelioma of the liver is a recently recognized and uncommon neoplasm of vascular origin. Few cases have been reported and the treatment remains therefore difficult to define. A relationship to oral contraceptive use has been suggested but no other toxic agents have been incriminated. We report here the first case occurring after a close contact with vinyl chloride. Because of wide hepatic destruction and serious portal hypertension with bleeding varices, the patient underwent orthotopic liver transplantation. After a disease-free interval of 20 months, he died from variceal hemorrhage and encephalopathy due to local tumor recurrence with portal thrombosis. Nevertheless, orthotopic liver transplantation remains the only hope of salvage when extensive liver destruction and life-threatening complications are present.