AbstractPurposeThe aim of this study was to evaluate the diagnostic accuracy of ¹⁸‐fluorodeoxyglucose‐positron emission tomography (¹⁸F‐FDG PET) and PET/computed tomography (PET/CT) in imaging primary and metastatic lesions in Ewing sarcoma (ES).MethodsPubMed, Cochrane, Scopus, and Web of Science were searched for relevant studies. Data concerning ¹⁸F‐FDG PET/CT diagnostic accuracy were extracted and then analyzed using Open Meta‐analyst software. Reported diagnostic accuracy outcomes included sensitivity, specificity, negative likelihood ratio (NLR), positive likelihood ratio (PLR), and diagnostic odds ratio.ResultsThirty‐one studies with a total of 735 patients were included in this meta‐analysis. The sensitivity and specificity of ¹⁸F‐FDG PET/CT were: 92.6% and 74.1% for total ES lesions, 96.7% and 68.3% for ES primary lesions, 76.1% and 92.4% for lung metastasis, 83.9% and 93.2% for bone metastasis, and 89.9% and 92.6% for ES recurrence, respectively.Conclusion¹⁸F‐FDG PET/CT is sensitive and accurate in diagnosing, staging, and detecting the recurrence of ES compared with non‐PET imaging. It has high accuracy for diagnosing recurrence of ES in bone metastases; however, CT remains a superior diagnostic method for detecting lung metastasis.