A 63-year-old man had a distal arch aneurysm combined with a circumflex retroesophageal right aortic arch, which is a rare form of vascular ring. By using combined median sternotomy and left thoracotomy under deep hypothermia and retrograde cerebral perfusion, total arch replacement with a vascular graft was successful. We describe our surgical strategy for arch aneurysm associated with an uncommon congenital vascular malformation.