This is the 28th case report of jugular foramen neurinoma in the world. A 24-year-old man was admitted to our hospital with complaints of dizziness and impaired balance. Neurological examination revealed IX, XI and XII cranial nerves and cerebellar involvements, e.g., Brun's-Cushing nystagmus, curtain sign on the left, weakness of the left sternocleidomastoid muscle and deviation of the tongue to the left, accompanied with atrophy. Choked discs and other signs of increased intracranial pressure were not recognized. There were no cerebellar symptoms except impaired balance and nystagmus. Lateral view of vertebral angiography showed that the posterior inferior cerebellar artery was displaced backward and upward, the basilar artery was imposed to the clivus, and the superior cerebellar artery was elevated. A-P view of vertebral angiography showed that the posterior inferior cerebellar artery was displaced to the left. Enlargement of the left jugular foramen was revealed especially by tomograms of horizontal section at the level of 0.3 cm below the external acoustic meatus and 0.5 cm behind the external acoustic meatus. The jugular foramen margins were smooth and somewhat sclerotic. We confirmed the diagnosis of the jugular foramen neurinoma on the left. Suboccipital craniectomy confirmed a huge tumor which covered the left jugular foramen and the bulk of which was in the cisterna magna. These findings were supposed to explain that the patient did not show increased intracranial pressure. The tumor was encapsuled with smooth and thin capsule. This tumor was totally removed and the postoperative course was uneventful. Histological diagnosis was neurinoma. We consider that this tumor arose on the IXth or XIth cranial nerve.