Esophageal atresia(EA) with a double tracheoesophageal fistula(TEF) is rare. It accounts for only 0.7% of all cases of EA and TEF. A male newborn weighing 2860g was born by normal vaginal delivery at 41weeks' gestation to a 27-year-old mother who had a normal pregnancy. But immediately developed recurrent choking and respiratory distress. Feeding tube was inserted and chest X ray showed the feeding tube coiled in the proximal pouch(T-2 level). With a preoperative diagnosis of EA and distal TEF, the thoracotomy was performed on the third day of life. At thoracotomy, TEF was proved to be a double fistula. Both fistulas were divided and an esophageal anastomosis was performed. The postoperative course was uncomplicated until the eighth postoperative day when a minor anastomotic leak. The patient was discharged at 103 days of age.