We aimed to share our very rare case with multiple congenital esophageal stenosis and its treatment. A 14-month-old girl presented with dysphagia, vomiting, and growth retardation, two esophageal strictures were detected in the esophagogram and esophagoscopic examination. While the proximal stenosis responded to balloon dilatation, the distal stenosis did not respond to dilatation and was perforated. Distal stenosis was treated with resection and anastomosis. Cases presenting with dysphagia and chronic vomiting should always be evaluated with esophagogram and endoscopy. Balloon dilatation should be the first choice in cases with stenosis. In cases that do not respond to dilatation, resection and anastomosis should be performed without insisting on dilatation.Keywords: Congenital, esophageal stenosis, multiple.