A localized thrombus involving the ascending aorta and arch rarely occurs in the absence of an underlying etiology such as chest trauma, atherosclerosis, a hypercoagulable state or instrumentation. A review of the literature between 1966 - 2003 yielded 38 reported cases of localized aortic arch thrombi, 21 of which were treated by surgical excision of the thrombus using different approaches. In this communication, we describe this clinical entity, with its diagnosis and management. The technical details that are important to ensure the safe conduct of the procedure are discussed.