We report a case of polymorphous hemangioendothelioma, a rare vascular neoplasm, arising in the liver of a 51-year-old woman. Microscopically, this neoplasm is characterized by a polymorphous blend of solid, primitive vascular and angiomatous areas in variable proportions. Polymorphous hemangioendothelioma is considered to be an intermediate-grade vascular neoplasm. In most of the reported cases, its occurrence has been in lymph nodes, but a few cases have been found in extranodal sites such as the mediastinum, spinal cord, and liver. Its classification among other soft tissue neoplasms is still controversial due to an insufficient number of cases to allow prediction of its biologic behavior. The aim of this article is to report a rare case of extranodal hepatic polymorphous hemangioendothelioma primary to the liver and to offer a summary of the reported cases in the literature, with emphasis on the differential diagnosis and diagnostic pitfalls in daily practice.