Primary intradural extraskeletal Ewing sarcoma is a very rare form of malignant neoplasm. Only few cases have been reported on the literature. Here, we report a case of a 14-year-old boy who had a chief complaint of pain and tingling in the right lower limb. The patient initially seemed to have a giant nerve sheath tumor but was eventually diagnosed with intradural extraskeletal Ewing sarcoma arising from the nerve roots of the cauda equine. The literature with regard to primary spinal intradural extraskeletal Ewing sarcoma is reviewed.
Keywords: Intradural; extraskeletal ewing sarcoma; nerve sheath tumor; spine.