Arterial complications of Behcet's disease rarely affect the thoracic aorta, and the incidence of aortic arch aneurysm is especially low. We present a patient who developed a rapidly expanding aneurysm of the distal aortic arch after 20 years of treatment of Behcet's disease. Emergency total arch replacement was performed with a favorable outcome. Graft anastomosis to the normal aorta involving all three layers, wrapping of the anastomotic sites with wide felt strips, as well as strict management of systemic inflammation are essential for prevention of late complications associated with graft anastomosis sites.