Primary malignant tumors of the aorta are extremely rare. Review of the literature indicates that there are nearly 100 recorded cases of primary malignant tumors of the aorta. The purpose of this article is to present an additional case of the primary malignant tumors of the aorta which initially has been misinterpretated as atherosclerosic disease. This aortic tumor was of endothelial origin and immunohistochemical studies classified the tumor as an epithelioid angiosarcoma. The treatment resulted in an abdominal aortic repair. MRI of the spine revealed multifocal metastatic disease of the axial skeleton and a subsequent chemotherapy was performed. The patient died 17 months after the initial diagnosis.