Persistent fifth aortic arch is a rare anomaly in congenital heart disease, which is often associated with aortic obstructive diseases. We report a 7-month-old infant diagnosed persistent fifth aortic arch (AA) with left ventricular dysfunction along with left bronchial malacia due to compression from own heart. Surgical repair was performed including AA reconstruction using the 5th-AA as an in-situ flap to enlarge the 4th-AA with end-to-end anastomosis, and external stenting for the left bronchial malacia. Postoperative courses were uneventful. On computed tomography, a reconstructed aortic arch without obstruction and an expanded left bronchus were seen.