Interrupted aortic arch (IAA) is defined as the loss of luminal continuity between the ascending and descending aorta and is classified based on the anatomic level of interruption. IAA is associated with a number of intracardiac anomalies with the most common being patent ductus arteriosus, ventricular septal defect, and left ventricular outflow obstruction. There is also a strong association between type B interruption and 22q11 deletion syndrome. The perioperative management of the neonate with IAA begins in the intensive care unit with optimization of end-organ perfusion and function. Survival depends on the prompt initiation of prostaglandin E1 in order to maintain ductal patency, careful management of the patient’s ratio of pulmonary to systemic blood flow (Qp:Qs), and a thorough understanding of the physiologic implications of the surgical plan, type of interruption, and associated syndromes and anomalies. This review will focus on the anatomy, physiology, and perioperative anesthetic management considerations specific to the management of IAA.