Cavernous angiomas of the internal auditory canal are rare lesions. The authors present a case of a 29-year-old lady with multiple infratentorial cavernous angiomas, whose sister had previously undergone surgery for a similar supratentorial lesion. She initially presented with an acute brainstem haematoma, secondary to a pontine cavernous angioma. Three years later she developed progressive right-sided sensorineural hearing loss and facial nerve paresis due to an internal auditory canal lesion. This was removed via the translabyrinthine approach and was found to be a cavernous angioma. This report underlines the multiple and dynamic nature of familial cavernous angiomas, as well as the importance of follow up to determine whether new symptoms are due to the enlargement of known angiomas or the development of new ones. As far as the authors are aware, this is the first report describing a cavernous angioma of the internal auditory canal in the context of familial and multiple infratentorial angiomas.