Petrous bone cholesteatoma, or cholesteatoma that extends beyond the middle ear and mastoid, represents a rare but destructive pathology. Diagnosis can be difficult before substantial morbidity is incurred, and patients can present with life-threatening complications. Determination of disease extent and the functional status of the facial nerve and cochleovestibular system are critical in surgical planning. Typically, surgery involves ablative procedures with the goal of complete disease resection given the low likelihood of preserved inner ear function. In experienced hands, disease control and facial nerve outcomes are favorable; however, disease recidivism is not uncommon and, thus, these patients require lifelong surveillance.