Disease of the apex of the petrous temporal bone, while rarely encountered, can present a unique challenge to the otologist. Lesions tend to be advanced at presentation, as massive bony erosion can remain asymptomatic. When symptoms occur, they reflect involvement of the neurovascular contents of the temporal bone. The earliest clinical features, such as headache, facial numbness and middle-ear effusion, do not immediately suggest the site or gravity of the underlying pathology. Anterior extension of disease may produce ophthalmoplegia and diplopia whilst posterior spread involves the lower cranial nerves, within the internal auditory canal, jugular foramen and hypoglossal canal.