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Introduction: Chordomas are slow-growing, low-grade malignant but locally invasive tumors which originate from embryonic remnants of the primitive notochord. Chordomas are principally midline tumors. In the neuraxis, chordomas are most commonly located in the sacrococcygeal region (50-55%), followed by the cranio-occipital region (25-30%). Case Report: A 71-year-old male has a left paraspinal tumor extending from C2 to C6 with bone erosion and left vertebral artery encasement. The tentative diagnosis before surgery was lymphoma or metastatic tumor. He underwent posterior cervical decompression with surgical debulking of the tumor to release the cord compression and posterior lamina screw fixation from C2 to C7 with allograft fusion and pathology confirmed the tumor to be chordoma. Conclusion: Due to the rare occurrence of chordomas extra-axially, these lesions have not earned a great deal of consideration in the clinical and radiographic differential diagnoses of extra-axial paraspinal lesions. An accurate preoperative diagnosis of chordoma is crucial, as survival is optimal when radical en bloc resection is performed at primary surgery.

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