Objective: To investigate the diagnosis and treatment of cardiac achalasia in children. Methods: The clinical data of 34 pediatric patients with achalasia admitted between September 2001 and September 2011 were retrospectively analyzed. All patients presented with typical manifestations, such as dysphagia, vomiting after feeding and “bird’s beak” narrowing of the distal esophagus on barium esophagogram. All patients underwent modified transabdominal Heller’s myotomy plus Dor’s fundoplication. Results: All the 34 patients were discharged from hospital 7 to 14 days after surgery and were able to tolerate semi-liquid diet before discharge. One patient was lost to follow-up while the other 33 patients were followed up for 2 months to 9 years and 7 months. The symptoms such as dysphagia, vomiting after feeding and gastroesophageal reflux were not present in any of the patients. The postoperative barium meal examination results showed that esophageal lumen in all patients were smooth, the narrow segment expanded and the barium passed easily into the stomach. Except for one child with trisomy 21, the growth and development of all the other cases were similar to their age-matched peers. Conclusion: Modified Heller’s myotomy has noticeable effectiveness on cardiac achalasia in children, and the additional Dor’s fundoplication is an important procedure for prevention of gastroesophageal reflux after esophagocardiomyotomy.