A two days old female baby was transferred to our hospital from local clinic due to respiratory difficulty. The baby was born at 38 weeks gestation through cesarian section in the clinic. At birth, the body weight of the baby was 2280 grams and the Apgar score at 1 minute and 5 minute were 5 and 7. The child had frothing about the nose and mouth, and regurgitated the first feeding almost immediately. Coughing and mild cyanosis were associated with regurgitation. She was diagnosed as distal tracheo-esophageal fistula with proximal esophageal atresia and transferred to our hospital for further evaluation and corrective surgery. She had no other congenital anomaly. The most common defect consists of a blind upper esophageal pouch and a fistula between the lower esophagus and trachea. The preferred surgical approach for treatment of the newborn with this disease is ligation of the defect and primary anastomosis of the esophageal segments by an extra-pleural approach. Proper placement of the tracheal tube is critical. It should be above the carina but below the tracheoesophageal fistula. Nitrous Oxide should be used with caution in a neonate without a gastrostomy, as diffusion of this gas into the distended stomach would be undesirable. The emergency operation was done under general anesthesia. She was tolerable anesthesia and operation. Special respiratory care and continuous antibiotic administration were done. She discharged 18 days later without significant pulmonary complication or sequelae.