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Cholesteatoma invading the internal auditory canal (IAC) is rare and usually exposes the facial and vestibulo-cochlear nerves functions to a serious threat. We reported the case of a 47-years-old patient having severe hearing loss in his left ear and facial paralysis in the affected side. Otomicroscopic examination of the left ear showed a whitish mass in the attic region covered by a reddish polypoidal tympanic membrane. Audiometric evaluation objectified the absence of response in the left ear. Temporal bone imagery revealed a left supra-labyrinthine petrous bone Cholesteatoma involving the IAC. A Transmastoid translabyrinthine (TL) with transcotic surgical approach has been programmed. Early diagnosis of cholesteatoma invading the IAC is important to prevent severe functional damage.