Tracheoesophageal fistula (TEF) and associated esophageal atresia (EA) in the neonate, presents during the first week of life. This congenital defect can be complicated by aspiration, respiratory distress, and other congenital anomalies. The knowledge and ability of the anesthesiologist to anticipate the challenges in managing neonates presenting for repair, plays an important role in their treatment and survival. Also, it is not uncommon for anesthesiologist to care for a patient later in life following repair of TEF. A familiarity with the immediate complications and long-term outcome and sequelae after TEF repair is important to ensure the best patient outcome.