Jugular foramen schwannoma (JFS) is a rare tumor that accounts for approximately 2%-4% of all intracranial nerve sheath tumors, originating from the Ⅸ, X, and Ⅺ pairs of cranial nerves. Patients usually develop between the ages of 30 and 60 years, and are more common in women. Hearing loss, tinnitus, ataxia and vertigo are the most common first symptoms. Sometimes a group of symptoms involving the jugular foramen area (including the IX, X and XI pairs of cranial nerve palsy) may manifest: it is called jugular foramen syndrome and includes hoarseness, dysphagia, loss of gag reflex, sternocleidomastoid and trapezius muscle paralysis, and loss of sensation in the posterior 1/3 of the tongue. A patient with jugular foramen occupancy was recently admitted to our department – Ms. Zhan had a paroxysmal dizziness and headache with paroxysmal deafness and occasional blurred vision without any obvious cause more than 20 days ago, and the cranial MR results from an external hospital showed an occupying lesion in the right pontocerebellar horn. In order to seek professional and systematic treatment, he came to our neurosurgery department for treatment. After the completion of relevant examinations, the diagnosis was: 1. right jugular foramen nerve sheath tumor; 2. obstructive hydrocephalus, and the operation was performed by posterior sigmoid sinus approach and right jugular foramen intracranial occupancy resection. Postoperatively, the patient did not show symptoms of posterior group cranial nerve injury and recovered well in all aspects. The postoperative MRI results showed complete resection of the occupancy. The nerve sheath tumor in the jugular foramen region originates from the neurohypophyseal cells of the linguopharyngeal, vagal paraglottic and hypoglossal nerves penetrating the jugular foramen, which is a slow-growing benign tumor with a low incidence. However, because of its deep location, various growth patterns and complex anatomical relationships, it is closely related to the posterior cranial nerves, brainstem and internal jugular arteries and veins, making surgery more difficult. Nerve sheath tumor in the jugular foramen area starts slowly and is often overlooked and misdiagnosed. Once similar symptoms appear in life, it should be actively examined and not ignored so as not to miss the best time for treatment.