Auditory nerve sheath tumor originates from the auditory nerve sheath and is a typical nerve sheath tumor. Since there is no involvement of the auditory nerve itself, the name of auditory nerve tumor is inappropriate and should be called auditory nerve sheath tumor. This tumor is one of the common intracranial tumors, accounting for 8.43% of intracranial tumors. Auditory nerve sheath tumors occur in middle-aged people, with peak incidence at 30-50 years of age, the youngest at 8 years of age, and the oldest at 70 years of age or older. Solitary auditory nerve sheath tumors occurring in the pediatric population are very rare. To date, there have been only case reports of auditory neuroma in children. The majority of swellings occur in the vestibular segment of the auditory nerve. A few occur in the cochlear region of the nerve, and as the tumor grows larger, it compresses the lateral surface of the pontine brain and the anterior border of the cerebellum, filling the pontocerebellar horn recess of the cerebellum. Most of the tumors are unilateral, but a few are bilateral; the opposite is true in the case of neurofibromatosis. This tumor is a benign lesion and does not become malignant or metastasize even if it recurs many times, and if it can be removed, it can often be cured permanently. However, because the tumor is adjacent to the important brainstem, surgical resection is still a great challenge for the neurosurgeon or otolaryngologist. With the improvement of neurosurgical methods and advances in neuroradiology, such as CT and MRI for early or qualitative diagnosis of tumor, brainstem auditory evoked potentials for early preoperative diagnosis, and the application of intraoperative electrophysiological monitoring and ultrasound suction, tumors can be safely removed and the function of facial auditory nerve can be preserved.