Aortic angiosarcoma is a rare malignancy. Clinical diagnosis is difficult, as the presenting symptoms mimic more common aortic lesions, particularly atherosclerosis. Dermatologists and dermatopathologists play a critical role in recognizing cutaneous metastases as a manifestation of this life-threatening tumor. We describe the fourth case of aortic angiosarcoma in the literature with initial presentation in the skin. (J Am Acad Dermatol 2000;43:930-3.)