Primary angiosarcoma of the aorta is a rare malignancy that is characterized by rapid proliferation and propensity for metastasis. It has been reported only 35 times in the surgical literature. This case report presents a 66-year-old man diagnosed with angiosarcoma of his native aorta 7 years after endograft repair of an abdominal aortic aneurysm. We then reviewed the world surgical literature for occurrence, tumorigenic studies, prognosis, and management of aortic angiosarcoma. Because native aortic tissue is retained after endovascular repair of an abdominal aortic aneurysm, the treating physician should have an awareness of this pathology and entertain the diagnosis as appropriate.