Meningiomas arising in the middle ear or presenting as a middle ear lesion are extremely uncommon and represent a diagnostic challenge. We report the case of a 79-year-old man, previously submitted to left ear cochlear implantation, treated at our Department for a suspected left chronic otitis media. Intraoperative findings and histology suggested a diagnosis of isolated left middle ear meningioma. The main radiological and pathological features of these lesions are discussed.