Tracheal agenesis is a very rare congenital airway anomaly of respiratory system with incidence of 1 in 50,000 newborns. The condition may be diagnosed antenatal if associated with Tracheo-esophageal fistula (TEF) presenting as Congenital High Airway Obstruction Syndrome (CHAOS). It may pose a great challenge to the attending neonatologist both in diagnosis and in establishing the airway soon after birth and associated with high mortality even with best possible facilities. Tracheal agenesis is difficult to detect prenatally. We report a case of tracheal atresia Faro type E associated with other congenital anomalies.