OBJECTIVE: We report the unique finding of petrous apex cholesterol granuloma associated with jugular paraganglioma (glomus jugulare). At the conclusion of this presentation, the participants should be able to: (1) Review the case of a patient that had a cholesterol granuloma in association with a jugular paraganglioma. (2) Understand the potential related pathogenesis. METHODS: The study design is a case report of a patient treated for a petrous apex cholesterol granuloma 20 years prior to presentation with a jugular paraganglioma. The pathogenesis of cholesterol granulomas and possible pathophysiology in relationship with paragangliomas will be discussed. RESULTS: At the time of initial presentation for the cholesterol granuloma, a jugular paraganglioma was not detectable on examination or CT scan. However, it is likely to have been present, but was not seen on the older imaging. The patient was treated for the cholesterol granuloma and returned 20 years later with signs and symptoms consistent with jugular paraganglioma. Microscopic bleeding from the paraganglioma may have lead to the formation of the cholesterol granuloma. CONCLUSIONS: The metachronous presentation of these two entities, which have not been reported previously in the literature, indicate the potential association of glomus jugulare with the formation of cholesterol granulomas of the petrous apex.