Epithelioid hemangioendothelioma of the liver is an extremely rare vascular tumor. It usually slowly evolves, affecting young adults without cirrhosis, and is characterized by multiple involvement of the liver, which contraindicates partial hepatectomy. This unusual tumor is often difficult to diagnose and differentiate from other lesions; its angiogenic nature can not be confirmed by radiography and conventional histologic examination. Its endothelial nature is based on ultrastructural studies and the demonstration of factor VⅢ-related antigen in tumor cells. We report one case of primary epithelioid hemangioendothelioma of the liver in order to describe its clinical, radiological and histological findings.