Nerve sheath tumors are benign tumors originating from peripheral nerve Schwann cells. Most intracranial nerve sheath tumors originate from the vestibular portion of the auditory nerve, and are commonly located in the pontine cerebellar horn region; axonal origins within the brain are rare, and intraventricular ones are even more uncommon. We report a case of nerve sheath tumor located in the fourth ventricle area. The patient was a 30-year-old woman. She had been walking unsteadily for half a year without any obvious cause, which was aggravated by spinning. Recently, hydrocephalus was detected by CT examination due to visual field defects. Physical examination: temporal visual field loss in the left eye, bilateral lower limb pathology sign (+), Romberg (±). He was admitted to our hospital for further diagnosis and treatment. Imaging manifestations: Cranial MRI scan showed a cystic solid mass in the area of the fourth ventricle, and the mass entity was located in the left pontine and cerebellar junction area in the transverse plane, which was poorly demarcated from the brainstem and cerebellar hemispheres, and the cystic portion was located in the position of the original fourth ventricle, which was pushed and flattened to the right, and the peripheral edema was not obvious, accompanied by supratentorial hydrocephalus. In the sagittal plane, the mass was seen to grow upward into the tetralogy of Fallot and the tetralogy of Fallot was pushed upward; the ipsilateral auditory nerve did not show obvious thickening. The solid part of the lesion and the cystic wall strengthened significantly after enhancement. The preoperative diagnosis was ventricular meningioma. The patient underwent tumor resection under general anesthesia. During the operation, the mass was found to be located in the cerebellar tonsillar region, with soft texture, clear boundary, cystic degeneration within the tumor, and the brainstem was pushed to the right by the tumor, and entered the jugular foramen in a downward direction. Postoperative pathology diagnosed as nerve sheath tumor in the tetralogy of fallot area.