Clear cell meningioma (CCM) is a rare subtype of World Health Organization grade II meningioma with a high rate of recurrence. Only a limited number of cases of distal recurrence or metastasis of CCM have been reported. We report a rare case of distal CCM relapse from an intracranial to intraspinal lesion. A 29-year-old female presented with diplopia and right partial sixth nerve palsy. Brain magnetic resonance imaging (MRI) revealed petroclival meningioma extending to the middle cranial fossa and right cavernous sinus. The tumor was subtotally resected and pathologically confirmed to be CCM. The patient underwent adjuvant radiotherapy targeting the remnant tumor site and intracranial tumor bed. At followup, MRI revealed a stable disease. Six years later, the patient developed lower back pain and bilateral buttock pain. Spine MRI revealed multiple intradural extramedullary tumors at the L2, L3, and L4-5 level, with the largest at the L3 level. The tumor at the L3 level was grossly totally resected in an en bloc manner. Histopathological findings revealed CCM. The patient underwent additional radiotherapy targeting the spinal canal and residual intraspinal tumors. CCMs can recur distally or metastasize throughout the whole neuraxis. Therefore, evaluation of the whole neuraxis is recommended.
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