Esophageal atresia with tracheosophageal fistula may be associated rarely with distal esophageal stenosis. Three patients are reported with this combination of esophageal anomalies. In addition the clinical and radiologic features of 24 patients previously reported in the literature are reviewed. Careful evaluation of the distal esophagus during postoperative contrast studies in patients with esophageal atresia should be obtained to exclude distal stenosis. The presence of unrecognized distal esophageal stenosis may lead to complications of postoperative anastomotic leaks, poor healing of the anastomosis, aspiration, and impaction of a solid food bolus proximal to the stenosis.