An 11-year-old boy presented to our pediatric pulmonology clinic with a 3-month history of atelectasis evident on his chest radiography. Breath sounds revealed fine crackles in the right lower zone and rhonchi in the upper zones. His initial pulmonary function test was compatible with restrictive pulmonary disease. Chest tomography revealed that the trachea, right intermediate, and middle lobe bronchi were narrowed by megaesophagus. Esophagogram determined dilatation of the esophagus and “bird-beak” sign in the esophagogastric junction but it was not sufficient to diagnose. Esophageal manometry which is the gold standard test for achalasia was performed and type 2 achalasia was diagnosed. His symptoms improved following Heller myotomy conducted together with Dor fundoplication. Although respiratory problems are more common in infants and younger children, atypical respiratory presentations may also occur during adolescence. Achalasia should be one of the rare differential diagnoses of pediatric restrictive pulmonary disease.