Ewing sarcoma (EWS) is primarily an osseous malignancy of childhood and young adults. Extraskeletal occurrence is less frequent and primary adrenal involvement is an even rare presentation. We present such a case of a 7-year-old boy diagnosed with adrenal EWS with associated venous thrombosis and pulmonary embolism detected on 18 F-fluorodeoxyglucose positron emission tomography-computed tomography scan.