Introduction: Tracheo-oesophageal fistula with or without oesophageal atresia is a congenital anomaly with an incidence of 1 in 3000-4000 births worldwide. Although congenital cardiac disease is the most frequent congenital anomaly, up to 50% of infants have TOF in addition to other congenital abnormalities. Case Report: At 3 day old the baby was planned for one lung ventilation with right thoracotomy + trachea-oesophageal repair in view of trachea-oesophageal fistula with oesophageal atresia. On examination, the general condition of the child was poor. He was tachypneic with a respiratory rate of 62/min along with chest retraction and nasal flaring. In the operating room, standard monitors were attached. Two intravenous line was secured with 24-gauge cannula on both dorsum of hands. Intravenous anesthesia drugs were loaded using insulin syringe according to baby’s weight. Induced with ketamine 4 mg and fentany l4 μg. After confirmation of bag and mask ventilation, succinylcholine 4 mg was given to facilitate end otracheal intubation. Trachea was intubated successfully with uncuffed endotracheal tube of internal diameter 3.0 mm confirmed by auscultation of chest and capnography. The child was ventilated with low tidal volume and high rate.  Anesthesia was maintained with oxygen, isoflurane and atracurium. The child was positioned in the right lateral position and thoracotomy was done. At the end of surgery, trial of extubation was done and due to insufficient  respiratory effort baby was shifted with ET tube in-situ to NICU. Conclusion: The anesthetic approach for neonates with TEF with or without EA should primarily concentrate on airway management and ventilation techniques. Maintaining airway patency, ensuring proper ventilation, and adequate oxygenation rely on effective communication between the pediatric surgeon and the anesthetist during the procedure Neonates are typically recommended to remain intubated endotracheally for a specific duration following surgery, necessitating the arrangement of an NICU bed in advance for these infants before definitive repair.