Esophageal atresia is a congenital disease that results in discontinuity of the esophagus and can occur with or without fistulous connections to the trachea. It is often associated with other anomalies and has multiple long-term health implications. Because of the complexity of care involved in affected children, it is important to diagnose this lesion as early as possible. Surgical repair via either anastomosis of the native esophagus or reconstruction using either the stomach or intestine if this is not possible remain the mainstays of therapy, although variations in technique prevail. In this review, we summarize the approach to diagnosis, preoperative management considerations, surgical approach, medical considerations, and long-term outcomes of infants with esophageal atresia.