Angiosarcomas are uncommon malignant tumors of vascular endothelium that represent less than 1% of all sarcomas. The epithelioid variant of angiosarcomas is exceptionally rare, and the gastrointestinal tract is rarely involved. Angiosarcomas mainly involve skin and soft tissue and rarely occur in breast, liver, bone, and spleen. We present the case of a 30-year-old man who underwent an upper gastrointestinal endoscopy for melena. A duodenal reddish polypoid lesion was found, which on microscopic examination turned out to be an epithelioid angiosarcoma. The immunohistochemical features of the lesion supported this diagnosis. The patient died eight months after the diagnosis. Epithelioid angiosarcoma is an aggressive variant of angiosarcoma and must be considered in the differential diagnosis of gastrointestinal tumors.