In recent years, the surgical correction of esophageal atresia with distal tracheoesophageal fistula (TEF) has become increasingly successful. However, there remains a group of high-risk patients with specific anatomical abnormalities in whom the mortality remains appreciable. These associated disorders include cardiac, renal, and chromosomal anomalies as well as severe respiratory distress syndrome. These factors, rather than low birth weight or early gestational age, are primarily responsible for surgical mortality. Preoperative telescopic bronchoscopy has been a useful adjuvant confirming the diagnosis, identifying unusual variants, and permitting the proper anatomic placement of the endotracheal tube. A Fogarty balloon catheter can be passed bronchoscopically into the distal TEF in patients with severe respiratory distress syndrome to occlude the fistula and facilitate effective positive pressure ventilation. Thirty-two patients were treated for esophageal atresia among whom 28 had esophageal atresia with distal TEF, three had esophageal atresia alone, and one had esophageal atresia with proximal TEF. The higher-risk group comprised those with severe respiratory insufficiency as evidenced by a room air paO2 of less than 60 mmHg; this group accounted for nine of the ten deaths in the total series. There was one late death following surgical correction. In summary, in the absence of severe respiratory insufficiency or associated life-threatening congenital anomalies, the results of surgical correction for esophageal atresia are remarkably good and survival in this group approximates 100%.