A case report of epithelioid adrenal angiosarcoma is presented. Tumor cells showed expression of cytokeratin, Factor VIII-related antigen, Ulex europaeus agglutinin-I, and vimentin. The patient also was found to have mesenteric fibromatosis (abdominal desmoid tumor) and an elevated serum level of estradiol. The authors discuss the unique appearance of these rare tumors, their relationship to hyperestrinism, and review the recent data in the literature showing cytokeratin expression by malignant epithelioid vascular tumors.