Background: Intradural spinal ependymomas are rare neoplasms derived from ependymal cells that typically arise within the spinal cord parenchyma. Extramedullary localization is exceptionally uncommon and often not considered in the differential diagnosis of spinal tumors. The tanicytic subtype, in particular, represents a distinct histological variant that further complicates clinical and radiological diagnosis.

Objective: This study aims to present a rare case of primary extramedullary cervical spinal ependymoma and to review the current literature on its clinical features, diagnostic strategies, histopathological characteristics, surgical management, and outcomes.

Methods: We conducted a comprehensive literature review and present a detailed case report of a 52-year-old male patient with a primary extramedullary cervical ependymoma. A descriptive research design based on published scientific data was used to contextualize the clinical presentation, diagnostic findings, surgical treatment, and postoperative outcome.

Results: The patient presented with a one-year history of cervical pain and progressive left-sided brachialgia. Magnetic resonance imaging revealed a well-defined, hyperintense intradural extramedullary lesion at the C5–C6 level. Surgical excision was performed under intraoperative neuromonitoring, achieving total removal of the tumor. Histopathological examination confirmed a tanicytic ependymoma (WHO Grade II). Postoperative recovery was uneventful, with complete resolution of radicular symptoms and no neurological deficits.

Conclusion: Primary extramedullary spinal ependymomas are exceedingly rare entities that should be considered in the differential diagnosis of intradural extramedullary lesions. Complete surgical resection remains the gold standard for treatment, often resulting in excellent neurological outcomes. Further studies are required to elucidate the pathogenesis, optimal management, and long-term prognosis of these tumors.