Interrupted aortic arch (IAA) is a rare congenital cardiovascular anomaly in which there is no direct continuity between the aortic arch and the descending aorta. A patent ductus arteriosus (PDA) usually feeds the descending aorta. But, as ductus constricts, IAA is lethal in nearly 100% of the cases, if untreated. The cause of death is a combination of greatly increased left to right shunt with increased pulmonary blood flow, which results in heart failure, pulmonary edema, and sequelae of the reduced perfusion of all structures distal to the interruption. During the noncardiac surgery in such patients, adequate managements with prostaglandin infusion, careful manipulation of the pulmonary-systemic blood flow ratio, inotropic support, and aggressive treatment of acidosis should be provided to ensure optimal outcomes of subsequent surgical reconstruction. We present the anesthetic experience of abdominal surgery in a neonate with a type A IAA combined with PDA and ventricular septal defect.