While primary cholesteatoma is rare, its presentation with only double vision merits extra attention. Apatient with primary cholesteatoma of the petrous apex where the only symptom was double vision is presented. Due to extreme medial localization there was absence of the common symptoms of hearing loss and facial paralysis. The patient was operated by the middle fossa intracranial approach and did well after surgery with no additional neurological deficit and no CSF (cerebrospinal fluid) fistulas. Extremely medially located primary petrous apex cholesteatoma are rare, can present with uncommon symptoms and can be successfully treated by the temporal intradural approach.