Ewing's sarcoma is a high-grade neuroectodermal primary bone tumor. This is the second primary bone tumor in children afterosteosarcoma and represents 4 to10% of cases. It can occur in all skeletal bones. However, the location at the facial bones is uncommon (1to2%) and extremely rare at the sphenoid sinus. We report the clinical results of a rare case of Ewing's sarcoma of the sphenoid with intraorbitaland intracranial extension.
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