A case of epithelioid hemangioma of the duodenum causing bleeding was encountered. A 32-year-old man was admitted because of anemia. Endoscopic examination revealed a submucosal tumor with central depression in the 2nd portion of the duodenum. It was considered to be the bleeding focus of anemia. A partial resection of the duodenum was performed. The macro- and microscopic examination of the specimen revealed an epithelioid hemangioma. Hemangiomas of the intestinal tract are rare, representing only 0.3% of all tumors of the intestinal tract, and among them, hemangiomas of the duodenum are extremely rare, representing only 3.4% of those of the intestinal tract. Most hemangiomas of the duodenum are cavernous hemangiomas or capillary hemangiomas. We report the first case of epithelioid hemangioma of the duodenum.