A 77-year-old female was admitted to our hospital due to congestive heart failure. Computed tomography and angiography showed a saccular aneurysm with aortopulmonary fistula in the aortic arch. The patient underwent total arch replacement and patch closure of the pulmonary artery in an urgent operation. She was discharged after uneventful postoperative course, although staged sternal closure and 5 days of respiratory management were needed. A few cases of aortic arch aneurysm with perforation to the pulmonary artery are operated with success, which depends on severity of congestive heart failure and urgency. Therefore, it should be diagnosed exactly and rapidly. In the operation, special care should be taken to avoid right heart failure and postoperative respiratory distress.