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Introduction: Facial nerve schwannoma is a rare benign tumor which may originate from any segment of the facial nerve. We present a case of a giant cystic facial nerve schwannoma that showed extension to the middle cerebral fossa, together with its clinical and radiological characteristics, and a short review of the related literature. Case Report: A 52-year old female patient attended with complaints of numbness in the right half of her face, and mild hearing loss. Examinations revealed House-Brackmann Grade 3 peripheral facial paralysis, sensorineural hearing loss of 30% in her right ear. She described three episodes of facial paralysis in the last six years. Cranial MRI and CT scan revealed a round cystic lesion of about 40x44x38 mm, located centrally and medially in the right temporal area. The surgical operation was performed and the lesion was totally extracted. The histopathological investigation reported the lesion as a schwannoma. Conclusion: Facial nerve schwannoma is a rare, benign tumor that occurs in different localizations. It may commonly be mistaken in prediagnosis and may be confused with other clinical states due to its clinical characteristics. It has to be kept in mind in the prediagnosis of patients with facial paresis and hearing loss.