Ewing sarcoma (ES) is the second most common primary musculoskeletal neoplasm in children and adolescents after osteosarcoma with a peak between 10 and 20. Rarely, the tumor develops in newborns or in adults over the age of 30. ES can affect either the appendicular or axial skeleton. Extraosseous ES is less prevalent. Clinical presentation is usually nonspecific. Radiography is the initial method for detection and imaging characterization of osseous ES. Computed tomography is used to assess bone destruction and characterization in anatomically complex areas, such as the spine and pelvis. MRI is the preferred imaging modality for local staging. The most characteristic finding on MRI is the presence of a large soft tissue mass. The definitive diagnosis of ES relies on a combination of histology, immunohistochemistry, and molecular testing. At initial presentation, 20%–30% of patients have pulmonary and/or skeletal metastases. The workup for pulmonary metastases includes a CT scan of the chest. PET-CT or WB-MRI is becoming standard for staging of osseous and nonosseous metastasis. The most important prognostic factor in ES is the presence or absence of metastasis. Follow-up imaging is performed during and after treatment, using the same imaging modalities as for initial workup.