Relevance. One of the leading positions among malignant tumors of the musculoskeletal system in children and adolescents has recently begun to be occupied by Ewing’s sarcoma (ES). The prognosis of patients suffering from this onconosology remains extremely unfavorable even in the absence of distant metastases and timely initiation of antitumor treatment. The authors present 10 years of experience in the management of pediatric and adolescent patients with ES in the pediatric oncology department of the National Medical Research Center of Oncology, Ministry of Health of Russia.Materials and methods. The study included pediatric and adolescent patients diagnosed with ES of I—IV stages of various localizations, who were treated in the Pediatric Oncology Department of the National Medical Research Center of Oncology, Ministry of Health of Russia in the period from 2009 to 2019. We analyzed 2- and 5-year overall (OS) and event-free (EFS) survival in patients who received combined and complex antitumor treatment for various forms of tumor dissemination according to the EURO-EWING 2008protocol, which included neo-and adjuvant polychemotherapy, including high-dose, radical surgical treatment, and also radiation therapy.Results. The median 5-year OS and EFS in the general group of the studied formulations was 60 and 17 months, respectively. The indicators of 2- and 5-year OS and EFS in patients of the general group were 80.6 % and 56.7 %, 38.6 % and 10.6 %, respectively. The volume of the performed antitumor treatment, as well as the form of the advanced tumor process, did not have a significant effect on OS and EFS indicators (p > 0.05). At the same time, the chances of developing ES in boys were statistically significantly higher by 2.2 times in comparison with girls (95 % confidence interval 1.1—4.3).Conclusion. The obtained OS and EFS indices indicate unsatisfactory results of anticancer therapy and extremely high aggressiveness of the biological behavior of the tumor, regardless of the form of spread of the tumor process.