Esophageal atresia with double tracheoesophageal fistula is a very rare anomaly and is difficulty to diagnose preoperatively. We treated a full term baby with esophageal atresia with double tracheoesophageal fistula. At the first operation, only the distal tracheoesophageal fistula was identified and ligated. When the upper esophageal pouch was opened, intermittent air leakages in sequence with positive bagging were noticed. However, intraoperative bronchoscopy did not identify a fistula in the proximal pouch, and the operation was completed with end to end anastomosis of the esophagus. On the 7th postoperative day, esophagography showed another tracheoesophageal fistula proximal to the esophageal anastomosis. A wire was placed in the fistula preoperatively under bronchoscopy. At the 2nd operation through the same thoracotomy incision the proximal fistula was identified and ligated. On the 12th postoperative day, esophagography showed neither stricture nor leakage.
