Primary sarcomas are rare tumors of the liver. Assessment of the diagnosis is difficult, particularly on percutaneous liver biopsy. Although liver transplantation (LT) can be indicated in selected cases of hepatic epithelioid hemangioendothelioma (HEHE) because of an excellent long term outcome, it is not advised for hepatic angiosarcoma (HAS) because of a high risk of early local or general recurrence rate after LT. We report the case of a young woman who underwent LT for a vascular hepatic tumor, of which some characteristics and the clinical presentation were suggestive of hepatic epithelioid hemangioendothelioma. However, the histological analysis of the explanted liver revealed hepatic angiosarcoma. We report an atypical clinical observation, which is especially interesting in that it illustrates the difficulty in distinguishing
the two forms of primary sarcoma of the liver.