Intramural hematoma of the aorta is a fatal disorder that remains poorly characterized. Recently, it has been accepted as a variant form of aortic dissection, where blood accumulates within the aortic media without the presence of an intimal tear. Clinically, it may present somewhat similar to dissection, and although optimal therapy remains controversial, current opinion supports surgery as the preferred method of treatment for intramural hematomas that involve the ascending aorta and aortic arch.