Purpose: Achalasia of the esophagus is a rare disorder in children; its symptom can mimic common childhood illnesses. In this study, the tactics of managing children with the corresponding pathology and evaluating the effectiveness of ongoing surgical treatment at the Filatov Children Hospital are considered. Materials: From 1991 to 2016, inthe Filatov Children Hospitalwas treated 39 patients with achalasia. Since 2011, all patients (27 cases) have undergone laparoscopic Hellercardiomyotomy with Dorfundoplication. The average age was 9.9 (4-15) years. The most frequent symptoms were vomiting (81%) and dysphagia (70%). Weight loss was observed in 48.1% of patients and chronic cough in 25%. Results: All children underwent laparoscopic Hellercardiomyotomy with Dorfundoplication. Intraoperative complication - damage to the mucosa of the esophagus occurred during cardiomyotomy in 2 cases (7.4%), which were cured during the laparoscopic procedure. There were no open procedures. Six (22.2%) required repeated intervention: pneumatic dilations (n = 2), balloon dilatation (n = 2) and re-surgery (n = 2). Conclusion: In our study, laparoscopic Hellercardiomyotomy in the case of achalasia of the esophagus is effective in 77.8% of children. We recommend this operation with Dorfundoplication, and believe that it is the operation of choice in the treatment of achalasia in children.