We present a case of an intimal epitheliod aortic sarcoma. Diagnosis was established after an aortic endarterectomy. The tumor was subsequently resected and an aortic graft was inserted. The patient underwent adjuvant chemotherapy. The patient is alive and free of metastatic disease at 48 months. Aortic tumors are rare, with an extremely poor prognosis and 1-year survival of 0-13%. The diagnosis is usually established postmortem, with metastatic disease being a common finding. The diagnosis should be suspected in patients with symptoms of nonatherosclerotic-related aortic occlusive disease or distal embolic events. Resection followed by adjuvant therapy and close follow-up can offer a prolonged survival for the patient with an extremely lethal aortic sarcoma.
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