Repair of interrupted aortic arch is associated with many intracardiac and extracardiac anomalies. The principles of interrupted aortic arch repair are mostly the same which include arch dissection, appropriate management of cardiopulmonary bypass and repair of the associated lesions. This chapter reviews the important steps of these operations to avoid operative complications such as cerebral ischemic injury, postoperative arch obstruction, recurrent laryngeal nerve injury, myocardial depression, and operative bleeding due to catheter disruption of the patent arterial duct.