UNLABELLED: Background: Petrous bone cholesteatoma (PBC) is a challenging condition involving the development of an epidermoid cyst within the petrous portion of the temporal bone. Advances in radiological imaging and skull base surgery have refined the management of PBC. Methods: An ambispective descriptive study was conducted on patients diagnosed with PBC between 2021 and 2024. Clinical and audiological evaluations were performed, and radiological imaging was utilized to assess disease extent. Surgical approaches were chosen based on cholesteatoma location and functional impairment. Postoperative outcomes, including complications and recurrence, were documented, and patients underwent regular follow-ups. Results: Ten patients with PBC were included in the study, majority of the cases were acquired cholesteatoma (8/10). Hearing loss was the most common presenting symptom and 3 patients had facial palsy at the time of presentation. Radiological features like carotid canal erosion and jugular bulb erosion were also evident in PBC. Surgical interventions included subtotal petrosectomy and radical mastoidectomy. Additional use of endoscopy ensured complete removal of cholesteatoma matrix. Postoperatively, improvements in facial nerve function were observed, with no recurrence noted during follow-up. Conclusion: Tailoring PBC management involves assessing lesion location, extent, and functional outcomes. Extensive disease may require aggressive surgical techniques, prioritizing hearing and facial nerve preservation. The endoscopic transsphenoidal approach is promising for petrous apex involvement, with benefits in morbidity and recovery. Vigilant postoperative monitoring is crucial for complication detection and long-term remission assurance. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s12070-024-05052-5.