Facial paraganglioma is an extremely rare tumor that originates from abnormal paraganglionic tissue situated in the intrapetrous facial canal. A review of the English-language literature shows that only 8 cases of facial nerve paraganglioma have been published. In each case the facial glomus presented itself sporadically, completely independent of any other form of paraganglioma. This study reports an intrapetrous facial glomus that occurred in a case of multiple paragangliomas with a hereditary pattern. To our knowledge, this is the first report of such a combination.