A case of primary fibrosarcoma of the thoracic aorta in a 57‐year‐old woman is reported. The clinical and pathologic features of this case are reviewed together with those of 12 cases of primary sarcoma of major systemic arteries recorded in the literature. Eight occurred in males and 5 in females. Macroscopically, the neoplasm may be polypoidal and intraluminal, intimal, or adventitial. Metastatic spread occurs with the arterial blood to sites peripheral to the primary growth, usually the lower half of the body, and the lungs are spared.