Spinal cord tumors are rare and fortunately affect only a minority of the population. These tumors are classified based on their anatomic location in relation to the dura mater and spinal cord as epidural, intradural extramedullary or intradural intramedullary. Intradural extramedullary Ewing's sarcoma is extremely rare. Here, we are reporting a case of intradural extramedullary Ewing's sarcoma. This is the fourth such case to be reported in English literature. A 26-year-old gentleman presented to us with low backache and progressive paraparesis from a spinal tumor. Magnetic resonance imaging showed multiple intradural, extramedullary mass lesions extending from the T11 to S2 level. Neither osteolytic nor osteosclerotic changes were seen in the vertebral bodies. Extraskeletal Ewing's sarcoma was diagnosed histopathologically. He was treated by surgery, local radiotherapy and chemotherapy. Two months after treatment, he developed a new skip lesion in the spine at T6-T7 level. The new lesion was treated with local radiotherapy and chemotherapy. Presently, the patient has completed treatment and is clinically doing fine. Intradural extramedullary Ewing's sarcoma is a rare aggressive neoplasm with high propensity for skip metastasis.
