The combination of left congenital diaphragmatic hernia (CDH) with esophageal atresia (EA) and distal tracheoesophageal fistula (TEF) is extremely rare and is considered highly lethal. The authors describe a premature neonate with this association, who is alive at 6 1/2 years of age. Temporary banding of the gastroesophageal junction and gastrostomy was performed concurrently with hernia repair and prosthetic abdominoplasty to enlarge the abdominal cavity. A right thoracotomy for ligation of the fistula, using extracorporeal membrane oxygenation (ECMO), was performed 13 days later. Complete repair of the esophageal atresia was accomplished 7 weeks after birth. The methods that have been suggested in the literature are discussed. The institution of ECMO at birth could allow a primary complete surgical repair of EA and CDH. Nevertheless, surgical management with staged repair, as described herein, can be useful.