Abstract
Extraskeletal Ewing sarcoma is a rare tumor mainly affecting young people, of poor prognosis with very high mortality rates especially in metastatic forms. It can affect different locations, without specific clinical signs, which delays the diagnosis. Imaging plays an important role for diagnosis, staging, preoperative assessment and surveillance. The diagnosis should be set early to a better management. We report a case of a 30-year-old man with a large extraskeletal Ewing sarcoma of the left thigh. The patient was initially treated with chemotherapy. Unfortunately, the tumor has increased in size making surgery impossible. The patient ultimately died of pulmonary metastases.