We present the rare case of a 74 – year – old woman with extensive intramedullary osteolysis of the petroclival skull base straddling both sides of the basi – occiput and basi – sphenoid. She presented with clinical features of recurrent spontaneous cerebrospinal fluid (CSF) otorrhoea despite three previous attempts at repair of the CSF leak. Recent advances in imaging techniques enable accurate radiological diagnosis of skull base lesions. We performed T1- and T2-weighted magnetic resonance (MR) images, a fluid attenuated inversion recovery (FLAIR) sequence MR, and a diffusion scan to study the characteristics of the skull base pathology. This revealed extensive osteolysis, with cystic spaces within the clivus and the petrous pyramid extending also to the basi – occiput. The leak was sealed using the technique of subtotal petrosectomy with obliteration of the eustachian tube and blind pit closure of the ear canal. The patient has been followed up for six months with no recurrence of symptoms.