Atypical meningioma originating from the spinal accessory nerve

BACKGROUND: Atypical meningiomas rarely originate from the spinal accessory nerve at the C1–C2 level. CASE DESCRIPTION: A 57-year-old female presented with a 1-month history of headache. The cervical MR revealed a well-demarcated intradural/extramedullary tumor compressing the spinal cord at the C1–C2 level that measured 12 mm × 10 mm × 25 mm. She underwent microsurgical tumor resection. Intraoperatively, the tumor was adherent to the spinal accessory nerve, rather than the dura mater. Gross total tumor resection was performed, and the pathology was consistent with an atypical meningioma. CONCLUSION: Atypical meningiomas rarely originate from the spinal accessory nerve. Gross total resection is the procedures of choice to mitigate the risk of tumor recurrence.