As a result of improvements in medical management, patients suffering from hemophilia have a life expectancy approaching that of the normal population. An increased life expectancy, however, brings an increased risk of developing age-related disorders. Several article have reported elective cardiac operations successfully performed on hemophiliacs. To our knowledge, there are no articles describing such patients undergoing emergent aortic surgery. Our report describes the successful management of an individual with hemophilia A undergoing emergent total arch replacement for acute aortic dissection (Stanford A). We used a continuous infusion of factor VIII concentrate during the perioperative period. The patient was discharged without bleeding complications.