Interrupted aortic arch is a rare congenital malformation, which is defined as a loss of luminal continuity between the ascending and descending aorta. Usually, there is a considerable distance between the ascending and descending parts of the aorta. According to the classification system of Celoria and Patton, three subtypes have to be differentiated. We describe a single-stage, extra-anatomic repair in an adolescent patient with a rare type C (the interruption is proximal to the left common carotid artery) interrupted aortic arch and his subsequent aortic valve replacement 19 years later.