Atresia of aortic arch (AAA) is very rare congenital anomaly and hemodynamically comparable with interruption of aortic arch (IAA). In general it is associated with pulmonary-ductus descending aorta trunk (PDDT) and its prognosis is extremely poor. 38-year old man complained of easy fatigability and hypertension on bilateral arms. Aortography showed interruption of blood stream at aortic isthmus and rich collateral blood flow to descending aorta. PDDT or intracardiac anomaly was not found. He was diagnosed IAA, type A of Celoria-Patton without PDDT. Alternatively echocardiography and magnetic resonance imaging revealed the anatomical continuity between the aortic arch and the descending aorta. Therefore he was finally diagnosed aortic arch atresia without PDDT. The prosthetic bypass grafting was performed between left subclavian artery and descending aorta.