Interrupted aortic arch is a rare congenital heart defect. Among patients with interrupted aortic arch, an interruption between the innominate artery and left common carotid artery (type C) is the rarest. Herein, we report the case of a neonate with aortic atresia, ventricular septal defect, type C interrupted aortic arch, and aberrant right subclavian artery who underwent Norwood stage I operation and staged biventricular repair. Due to aortic atresia, coronary artery perfusion was retrograde from the right common carotid artery. The surgical technique and special considerations for cardiopulmonary bypass are presented.