Esophageal stenosis caused by an intrinsic congenital deformity is uncommon in infants and children. The main forms of stenosis are congenital esophageal web congenital stricture caused by tracheobronchial remnants, and congenital idiopathic muscular hypertrophy. The authors report on two patients who were successfully treated and managed after being diagnosed as having upper esophageal stenosis. One patient underwent resection of the web and primary anastomosis of the esophagus and was discharged 6 days after surgery. After 1 year, this patient has had no symptoms of dysphagia or other postoperative difficulties. The second patient underwent balloon dilatation of the esophageal stricture and was discharged on the day of surgery; however, this patient required numerous repeat dilatations.