Congenital esophageal stertosis is a narrowing of esophageal lumen that is present at birth, and may be asymptomatic in the neonate. Stenosis of the lower esophagus is a very rare form of esophageal obstruction. Three types of congenital esophageal stenosis have been described: fibromuscular stenosis, membranous webs, and tracheobronchial remnants. Fibromuscular stenosis and membranous webs respond to dilation, but must be distinguished from strictures caused by peptic esophagitis. Tracheobronchial remnants generally require surgical therapy. We report a 5-year-old girl with congenital esophageal stenosis, who presented with persistent dysphagia and poor weight gain. An esophagogram showed stricture of lower esophagus with proximal dilatation above esophagogastric junction. She was successfully treated with endoscopic balloon dilation.