Congenital esophageal stenosis (CES) is a narrowing of the esophageal lumen from birth. Three types of CES have been described; tracheobronchial remnants (TBR), membranous web (MW), and fibromuscular stenosis (FMS). We reviewed the clinical features and the surgical outcome of 14 patients, pathologically confirmed as CES. Nine patients had TBR, 3 FMS, and 2 MVV. The mean age at operation was 3.8 years. Five patients were boys and 9 girls. Four patients had other congenital anomalies. Segmental resection of the lesion and end to end anastomosis was utilized in all cases except one who underwent myotomy. The stenotic segment was located at the distal esophagus in all patients. There were 8 complications in 6 patients, but no mortality. The mean follow-up period was 68 months. There were no feeding problems but 3 patients had minor gastroesophageal reflux. Our result indicates that segmental resection and anastomosis is a satisfactory surgical procedure in the management of CES.