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Primary intracranial epidermoid carcinoma is an extremly rare sequela of epidermoid cysts. The authors had experienced a case of primary intracranial epidermoid carcinoma in the right cerebellopontine angle. Here, we report clinical, radiological, and histopathological features of this neoplasm with review of literatures. Key words:Epidermoid cyst;Epidermoid carcinoma;Cerebellopontine angle.