Neuroglial ectopia is a rare entity of undetermined clinical significance. Here, we report a unique case of neuroglial ectopia of the vestibular nerve. A 27-year-old pharmacy student with a previous radiological diagnosis of vestibular schwannoma presented to our clinic for surgical evaluation. Magnetic resonance imaging (MRI) of the brain revealed a 17-mm T1 hypo- to isointense, T2 iso- to hyperintense, poorly enhancing left cerebellopontine angle mass extending into the left internal auditory canal compatible by imaging with a vestibular schwannoma. The lesion was resected under MRI guidance. The frozen specimen came back as a benign hypocellular lesion. Histological assessment revealed a peripheral nerve engulfed by glial fibrillary acidic protein-positive, S-100-negative cells, suggestive of neuroglial ectopia. There was no evidence of schwannoma. The main concerns were benign neoplasm with potential for progression or sampling artifact. The patient had an uncomplicated hospital course. This case report demonstrates an unusual case of neuroglial ectopia of the vestibular nerve. The differential diagnoses for a poorly enhancing cerebellopontine angle mass should include neuroglial ectopia.

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