The interrupted aortic arch is very rare and occurs in about 1% of the infants with critical congenital heart disease. Perfusion distal to the interruption is dependent on the patency of the ductus arteriosus. Unrecognized and untreated cases have a median age of death at 4–10 days of life. Management of the interruption requires timely diagnosis, use of prostaglandin E infusion immediately after birth to ensure end-organ perfusion, and eventual surgical repair of the interruption. Very rarely there have been cases of patients surviving longer with significant collaterals supplying the lower half of the body. High-quality echocardiography can reveal types of interrupted aortic arch and associated cardiac lesions. Cardiac CT angiogram has played a very important role in delineating details of the interrupted aortic arch, absence/presence of collaterals, intersegmental distance to plan appropriate surgical approaches, and follow-up study to evaluate recurrent coarctation postoperatively.