Epithelioid hemangioendothelioma (HEE) is an unusual malignant neoplasm of vasculo-endothelial origin, arising in soft tissues, lung and liver. One case of hepatic HEE is reported in a 32 year-old man wit an isolated multinodular hepatomegaly. The diagnosis was made by the histologic examination of biopsy specimens. At the time of diagnosis, there was evidence of pulmonary metastasis. However, an orthotopic liver transplantation was performed. The tumor cells were immunoreactive with factor VIII-related antigen, BNH9 and vimentin. There was no expression of cytokeratin and epithelial membrane antigen. The patient is alive and well 24 months after. The thoracic X-ray are unchanged. This case is compared with the 84 others cases of the literature. Fourteen experienced a favorable outcome after transplantation. Extra-hepatic dissemination doesn't contra-indicate the graft. But hilar vascular involvement may be correlated with worse prognosis.