Rationale:Esophageal achalasia is characterized by impaired relaxation of the cardia and dilation of the intrathoracic part of the esophagus. We describe the late presentation of achalasia in an 11-year old girl.Patient concerns:She suffered from recurrent pneumonia, obstructive bronchitis, and problems with swallowing solid food. Her family noted a wet pillow in the morning.Diagnoses:This case report describes the typical symptoms of achalasia in children in order to facilitate earlier diagnosis of this rare disease. Our patient was admitted to a pediatric hospital for treatment of severe pneumonia, low-grade fever, and pancreatitis. A computed tomography (CT) scan of the thorax showed massive dilation of the esophagus and infiltration and partial atelectasis of the right lung. An upper gastrointestinal contrast study confirmed massive dilation of the esophagus and stenosis at the level of the cardia.Interventions:We performed laparoscopic Heller myotomy combined with Dor fundoplication. Bronchoscopic lavages were conducted in the pre- and postoperative period to relief obstruction of bronchi by purulent mucus secretions.Outcomes:A further upper gastrointestinal contrast study demonstrated patency of the cardia and fast propulsive movement of contrast agent into the stomach. At follow up 2 months after the operation, the girl had gained 3 kg of body weight, and her respiratory, gastrointestinal, and swallowing symptoms had subsided. At follow-up 12 months after the operation, no recurrent symptoms of achalasia were recorded.Lessons:Late presentation of achalasia in children can mimic respiratory and gastrointestinal diseases. Laparoscopic Heller myotomy combined with Dor fundoplication is feasible and advisable in children suffering from achalasia of the cardia.