The association of pure esophageal atresia, duodenal atresia, and unilateral lung agenesis has not been reported previously. Here the authors present a case of a newborn with this constellation of anomalies that underwent staged repair. The primary principle guiding treatment was the avoidance of iatrogenic injury to the single lung. Therefore, the order of operations proceeded as follows: (1) placement of a decompressing gastrostomy tube, (2) repair of the duodenal atresia, and (3) repair of the esophageal atresia. The congenital closed loop obstruction caused by the esophageal and duodenal atresias was beneficial in that it resulted in growth by stretching of the distal esophagus, allowing a tension-free primary repair of the esophageal atresia.