We describe a forty-six-year-old man who died of disseminated angiosarcoma ten months after a radical nephrectomy and postoperative radiation therapy for a renal angiosarcoma. We found 3 other well-documented reports of renal angiosarcoma, all in men. Two of the 3 cases previously reported also had a rapidly fatal course with hematogenous spread of the neoplasm following nephrectomy. Angiosarcoma of the kidney, although rare, is apparently a highly malignant fatal neoplasm, and effective therapy remains unknown.