We report a case of a petrous apex cholesteatoma which was managed with a wholly endoscopic permeatal approach. A 63-year-old Caucasian male presented with a 10-year history of right-sided facial palsy and profound deafness. On examination in our clinic, the patient had a grade VI House-Brackmann paresis, otoscopic evidence of attic cholesteatoma behind an intact drum, and extensive scarring of the face from previous facial reanimation surgery. Imaging review was suggestive of petrous apex cholesteatoma. An initial decision to manage the patient conservatively was later reviewed on account of the patient suffering recurrent epileptic seizures. A wholly endoscopic permeatal approach was used with successful outcomes. In addition to the case report we also provide a brief description of the technique and a review of the relevant literature.