Background: The aim of this retrospective study was to highlight the role of FDG PET/CT in the assessment of tumor response to preoperative chemotherapy in pediatric sarcoma. Eighteen patients were included in our study: 13 patients were males and 5 were females ranging between 1 and 18 years with a mean age of 13.3 years. The patients had pathologically proven osseous or soft tissue sarcoma. All patients underwent sequential 18F-FDG PET/ CT before (PET-CT1) and after (PET-CT2) neoadjuvant chemotherapy. Maximum standardized uptake value (SUVmax) was measured in the primary lesion on PET/CT1 (SUV1) and PET/CT2 (SUV2). After surgery, the effects of neoadjuvant chemotherapy were evaluated histopathologically: ≥ 90% necrosis indicated was considered a good response and < 90% necrosis was considered a poor response. The correlation between SUV2 and the histologic response was assessed. Results: The sensitivity, specificity, positive predictive value, and negative predictive value of SUV2 for assessment of treatment response were 100%, 91.67%, 85.71%, and 100%, respectively. The overall accuracy was found to be 98.3%. Conclusions: 18F-FDG PET/CT provides a reliable non-invasive diagnostic tool in assessment of response to preoperative chemotherapy in pediatric sarcoma.