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Meningiomas account for 13 to 37% of all primary brain tumors, with 15% of all intracranial meningiomas arising in the posterior fossa. Patients with jugular foramen meningiomas (JFMs) may present with vague, non-specific vertiginous symptoms and sensorineural hearing loss (SNHL). Recently, the author encountered a unique case of JFM with symptoms similar to those of Ménière’s disease and those of delayed endolymphatic hydrops. The patient discussed in the present case is a 17-year-old woman with a several month history of right SNHL and recurrent attacks of vertigo. The initial diagnosis of vertigo was later diagnosed as a JFM of the clear cell subtype, which is a remarkably rare pathological finding among intracranial meningioma. This study reports a case of JFM of the clear cell subtype in addition to a review of the literature. Korean J Otorhinolaryngol-Head Neck Surg 2016;59(10):746-53