Background:Congenital esophageal stenosis (CES) is an uncommon condition presenting with swallowing problems. It has been classified into three types. Clinically, the condition is manifested as dysphagia. This is more common after introduction of semisolid to solid foods. We present the management strategy of this uncommon entity.Methods: This study ranged from January 2009 to January 2015. Six patients of CES were included in the study. The patients were diagnosed by contrast swallow study and upper gastrointestinal endoscopy. The initial management included esophageal dilation under fluoroscopy guidance. In case of failed dilation, thoracotomy, resection of the stenotic segment and primary esophageal anastomosis was performed. If required, antireflux procedure was added.Results:In 4 patients, the stenosis was in middle esophagus. Of these, one had associated esophageal diverticulum. In 2 patients, the stenosis was in the lower esophagus. These patients also underwent antireflux surgery. After the surgery, all patients were able to swallow properly. There was no problem in the follow up except in one patient, who needed dilation in the follow up. He responded to the dilation.Conclusions:High index of suspicion is needed to diagnoses this condition. Dilation may not be successful to treat all type all type of CES, for whom surgery will be needed. Long term outcome may be satisfactory.