Epithelioid hemangioendothelioma (EH) is a rare vascular neoplasm that was first described by Weiss and Enzinger [1982]. The natural history and clinical course of EH is largely unknown given its rarity, but its malignant potential is thought to range between that of a benign hemangioma to a malignant angiosarcoma [Woodall et al. 2008;Mehrabi et al. 2006;Uchimura et al. 2001]. Primary disease can involve soft tissue and visceral organs to include heart, spleen, lung, bone, and lymph nodes [Woodall et al. 2008;Mehrabi et al. 2006]. Primary malignant hepatic epithelioid hemangioendothelioma (HEH) is an exceptionally rare tumor with a reported incidence of less than 0.1 per 100,000, with less than 440 published reports in the literature [Mehrabi et al. 2006]. The exact etiopathogenesis of HEH is unknown but possible etiologic factors have been described to include oral contraceptives, vinyl chloride, asbestos, hepatic trauma, viral hepatitis, primary biliary cirrhosis, and alcohol consumption [Mehrabi et al. 2006].