Primary arterial neoplasms are rare lesions which have been most frequently associated with local or constitutional symptomatology, and with distal embolization. Perirenal aortic disruption with pseudoaneurysm formation due to an intimal sarcoma adjacent to a previously placed prosthetic graft is reported in a 66-year-old man. This case supports the premise that the presence of a vascular prosthesis might result in the induction of an arterial wall malignancy. This should be considered when an intraluminal mass is identified in the absence of other arterial pathology. Although the prognosis of these tumors is poor, their preoperative recognition may enhance treatment outcomes.