Intradural extramedullary tumors are predominantly benign [1]. Schwannomas and meningiomas are the most frequently encountered diagnoses, with ependymomas following closely [1]. Meningiomas, more prevalent in women, typically manifest in the thoracic region, whereas schwannomas are commonly located in the cauda equina [2]. Ependymomas and paragangliomas are exclusively localized to the cauda equina [2]. Initial clinical manifestations often include lower limb weakness, radicular pain, lower back pain, or sphincter disturbances, which typically develop gradually and insidiously. Metastases and other primary tumors are considerably rarer occurrences [1]. Rare primary spinal tumors include solitary fibrous tumors, lipomas, paragangliomas, and inclusion cysts such as dermoid and epidermoid cysts [3]. An exceptional inclusion in this category is extraosseous Ewing sarcoma, as exemplified by our case. Given the variability in histology, precise diagnostic and therapeutic strategies are of paramount importance.

The Ewing sarcoma family of tumors affecting the spinal cord represents a rarity [4], primarily originating from osseous Ewing sarcoma-induced compression of the spinal cord, typically originating within the vertebrae [4]. Intradural involvement of extraosseous Ewing sarcoma is particularly atypical, and its consideration during lesion assessment is not commonplace.

This report details a case involving a 66-year-old male who presented with progressive low back pain and sciatica attributed to an intradural Ewing sarcoma. Diagnosis was confirmed through identification of the characteristic EWS fusion transcripts. This case contributes to the existing literature by highlighting the uncommon nature of this diagnosis when encountering an intradural extramedullary lesion.