Primary malignant tumors of the aorta (PMTAs) are an extremely rare clinical entity. In most cases, the diagnosis is made either postmortem or late in the course of disease due to a low index of clinical suspicion and presenting symptoms that mimic far more common athero-embolic processes. Due to the intravascular origin of these tumors, peripheral and mesenteric embolic events represent a major source of morbidity and mortality. We present the first reported case of a patient with a diagnosed PMTA treated with an endovascular aortic stent for the purpose of embolic prophylaxis. The presentation, diagnosis, classification, and management of PMTAs in the world literature are also reviewed.