Abstract An epithelioid hemangioendothelioma arising from the innominate vein in the anterior superior mediastinum of a 62-year-old man was treated by wide local excision followed by radiotherapy. This clinicopathologic entity is a rare cause of mediastinal mass and is characterized by an intermediate malignant potential. To our knowledge, very few cases have been reported in the mediastinum; those cases are listed in. this report. Gross and microscopic distinguishing features of epithelioid hemangioendothelioma, as well as differential diagnosis, are reviewed from the literature. Finally, clinical behavior is discussed and treatment options are suggested.