The treatment of Ewing’s sarcoma in children remains difficult and unpredictable. Especially in the group with lung metastases (PM), the role of metastectomy has not been still clearly defined. The study aimed to define the best management strategies in children with PM. Clinical factors (age, localization, response to chemotherapy (CHT) of the primary tumor (PT), R0 and R1 primary resections, metastectomy, the use of radiotherapy (RT), autologous hematopoietic stem cell transplantation (aHSCT) were analyzed in the group of 137 patients (pts) with PM (85-isolated, 52-lungs and extrapulmonary (ExPM)) treated during the period 1998-2024. The mean f-up was 103.8 months (8.19 years). Descriptive statistics were used to summarize the characteristics of a data set. Survival curves were obtained by using the Kaplan-Meier method. Log-rank test was used to identify the prognostic significance of clinical factors. Pts with isolated PM (85 pts) had better OS than pts with PM and ExPM (p=0.000003). The good histological response of the PT (<10% viable tumor cells) to CHT was only the EFS-predictive factor in this group (p = 0.002). Pts with isolated PM and good histological response treated or not with metastectomy had better EFS than pts with poor response to CHT (p = 0.001). Pts treated with thoracotomy had significantly better OS (p = 0.001) than pts treated with RT of metastases. Good response to CHT of the PT had the same impact on OS (p = 0.001) in this group. A similar trend was observed in the groups with good response to CHT treated with thoracotomy and RT of metastases vs RT only (OS – p = 0.02). Pts with poor response to CHT and isolated PM treated with RT without thoracotomy had the worst prognosis (p = 0.02). In the group of 52 pts with PM and ExPM RT of PM and extrapulmonary metastases (p = 0.04) was EFS-predictive factors. Conclusions Metastectomy of isolated PM may improve the results of patients with good histological response to CHT of the PT. Patients with simultaneous pulmonary and ExPM achieve worse treatment results. The use of RT on PM and extrapulmonary metastatic lesions seems to improve outcomes.