In a 10-year period, 22 neonates with esophageal atresia (EA) and tracheoesophageal fistula (TEF) required high pressure ventilatory support soon after birth because of respiratory distress syndrome (RDS). Eleven of the 22 or 50% survived overall, but if the 5 patients who died before definitive surgical repair could be attempted are excluded, 11 of 17 or 65% survived. More importantly, 4 of 7 (57%) patients who had gastrostomy performed first survived while 7 of 10 (70%) who had fistula ligation performed first survived. The difficulties with intraoperative management of those who had gastrostomy performed first were even more impressive. Our experience leads us to conclude that patients with EA and TEF with severe RDS who require high pressure ventilation preoperatively represent a group of patients who require special consideration. The danger to such patients with increased pulmonary resistance is not gastric distention but sudden loss of intragastric pressure. In the presence of poor lung compliance, the upper gastrointestinal tract functions in continuity with the tracheobronchial tree. A sudden loss of intragastric pressure, as with placement of a gastrostomy tube, results in an acute loss of effective ventilating pressure. Resuscitation of such a patient is not possible until leakage from the esophagus is controlled by ligation of the fistula or transabdominal occlusion of the distal esophagus. Placement of a Fogarty catheter into the fistula via a bronchoscope is effective but may not be feasible in every case. Early thoracotomy and ligation of the fistula in patients with progressive RDS provides immediate improvement in ventilatory efficiency and relief of gastric distention.