Pulsatile tinnitus, hearing loss, lower cranial nerve deficits, and radiographic evidence of a vascular lesion of the jugular foramen have been considered diagnostic of a glomus jugulare tumor. Angiographic evidence of a blood supply from the external carotid artery system, including the ascending pharyngeal artery, further substantiates this diagnosis. This diagnostic algorithm for tumors of the jugular fossa is usually followed by either a surgical exenteration of a presumed glomus jugulare tumor via an infratemporal fossa approach or radiation therapy in selected patients. Pre-treatment biopsy of such lesions is typically not done, nor is it recommended widely in the literature. As demonstrated in this report, a number of lesions, including hemangioperi-cytoma and extramedullary plasmacytoma presenting in the jugular foramen can mimic glomus jugulare tumors in all aspects of their clinical and radiographic presentation. Omission of a pre-treatment biopsy can lead to a treatment plan appropriate for glomus tumors but suboptimal for these rare pathologic entities. A pre-treatment biopsy of lesions of the jugular foramen by exploratory tympanotomy or postauricular mastoidotomy provides a pathologic diagnosis on which to base treatment of lesions of the jugular foramen, without adding substantial morbidity or decreasing the chances of cure.