Esophageal involvement is frequent in Down syndrome. We report a case of dysphagia in a 21-year-old patient with Down syndrome and repaired esophageal atresia. Radiology, endoscopy, and manometry showed typical features of achalasia. The patient was treated first by botulinum toxin injection and afterwards by Heller myotomy with good result. The role of motor disorders associated with esophageal atresia or with primary achalasia in this patient is discussed.