Primary aortic mural thrombus(PAMT) is a relatively rare clinical disease with unclear pathogenesis. In addition to hypercoagulability as a recognized risk factor, other related factors have been mentioned in the literatures. Aortic isthmus is the most common site of thrombosis, which may be related to its anatomical characteristics and hemodynamics. The onset of this disease is insidious, but the complications are dangerous. At present, the best treatment strategy for this disease is not clear, and simple anticoagulant therapy, aortic open surgery and endovascular treatment have been reported successfully. This article reviews the research progress of primary aortic mural thrombosis and reports as follows.