Neurofibromatosis type 2, also known as NF2, is an autosomal dominant disorder with diverse clinical manifestations, typically represented by bilateral auditory neuromas. According to a British study, a total of 64 cases of NF2 were identified in the northwest of England over a decade, with an annual incidence of 1 in 2355,000. In a large population-based sample, approximately 50% of NF2 patients have a family history, suggesting that NF2 is a genetic disease. The incidence of NF2 is not high, but the patients are often adolescents, and the disease is complex and difficult to treat. We had a patient who was 23 years old, a young woman, who had surgery for a right-sided auditory neuroma at the age of 18, and who had postoperative hearing loss and right-sided facial paralysis. At the time of consultation, she had already experienced hearing loss in her left ear and needed to wear a hearing aid, and an MRI revealed a small auditory neuroma on the left side. In such a patient, she should be advised to learn lip reading and sign language as early as possible and be followed up to observe the tumor growth and hearing loss on the left side. The probability of her child having NF2 is 50% and therefore fetal genetic diagnosis should be performed during pregnancy. Patients with NF2 often have a family history of the disease, which most often develops in childhood or adolescence. The disease progresses rapidly, with eventual bilateral hearing loss, and without treatment, most patients die before the age of 20. Bilateral auditory neuromas are the most common manifestation of NF2, but the size and growth rate of bilateral auditory neuromas are not uniform, and the size of the tumor does not correspond exactly to the hearing condition. The most common clinical presentation is unilateral or bilateral progressively increasing sensorineural deafness, which may be accompanied by tinnitus, vertigo, or balance disturbances. Skin and spinal cord tumors are also common occurrences. Ocular symptoms are also common, clinically manifested by amblyopia, strabismus and cataracts. MRI plays an important role in the diagnosis of NF2, and MRI findings of tumors are the gold standard for diagnosis. MRI of the brain, internal auditory tract and spine is mandatory for patients with clinical suspicion of NF2, and enhanced MRI can detect tumors as small as 1-2 mm. Treatment of NF2 must be noted to have different points from unilateral auditory neuroma. The principle of treatment for unilateral auditory neuroma is based on total resection of the tumor and preservation of the facial nerve as the main goal, on top of which hearing is preserved as much as possible; unilateral hearing loss does not significantly affect the patient’s quality of life. In contrast, for bilateral auditory neuroma, the hearing factor must be emphasized; if bilateral hearing is lost, it can seriously affect normal working life. The surgical approaches for bilateral auditory neuroma can be generally divided into two categories: surgeries that attempt to preserve hearing and surgeries that do not preserve hearing. The surgical approaches that attempt to preserve hearing include the middle cranial fossa approach and the posterior sigmoid sinus approach (suboccipital approach), but in practice, when the tumor size exceeds 20 mm, hearing preservation is basically impossible. The surgical approach that does not preserve hearing refers to the vagus approach, which is the best surgical option for tumors with hearing loss and tumors larger than 20 mm. Regardless of the surgical approach, the integrity of the cochlear nerve should be preserved as much as possible to allow for cochlear implantation. In patients with bilateral auditory neuromas, treatment options should be carefully chosen. For patients with small bilateral tumors, a follow-up plan is preferable. This is done by annual MRI examinations to understand the rate of tumor growth, close observation of hearing loss levels, and informing the patient and family of the surgical options available and the pros and cons of surgery. Each family’s condition and each patient’s personality and psychology are different. When there are multiple options and it is difficult to distinguish the advantages and disadvantages of different options, treatment should be individualized rather than procedural. Radiation therapy was once considered as an alternative to eliminate the risk of surgery and improve the quality of life of patients. However, the complications of radiotherapy deserve attention, including hydrocephalus and perineurial edema. In particular, it is important to note that auditory neuroma itself is a benign tumor, while some patients will experience malignant changes in auditory neuroma after radiation therapy, and spinal neuroma is 10 times more likely to become malignant. About 10% of patients have recurrence after radiotherapy. After radiotherapy, the tumor adheres closely to the surrounding tissues, making surgery significantly more difficult, and not only is it impossible to preserve hearing, but facial nerve function is also greatly affected. For radiotherapy, our opinion is that surgery should still be the primary treatment option, and radiation therapy can be considered for those patients who cannot tolerate surgery. There are two options for postoperative hearing rehabilitation in NF2 patients: cochlear implantation (CI) and auditory brainstem implantation (ABI). The majority of patients who undergo CI outperform those who undergo ABI, according to a combination of available studies. In patients with NF2, where bilateral hearing loss is the result of the disease process, CI can restore hearing, either by preserving the cochlear nerve at the time of tumor surgery or by implanting it in two stages, or by performing CI on the side of a small tumor where hearing loss is not life-threatening. NF2 is an autosomal dominant disorder with an early age of onset and irreversible hearing impairment, so early detection and timely intervention is important, which requires genetic diagnosis and genetic counseling. Familial NF2 patients have a 50% chance of passing the mutation on to the next generation, and 95% of mutation carriers will develop bilateral auditory neuromas. The rate of positive genetic tests for first-degree relatives of NF2 patients is only 75%, but the rate can be increased to 100% by screening NF2 patients for the mutation locus and performing genetic screening of first-degree relatives based on the locus. In conclusion, NF2 is a hereditary, systemic disease and is a complex problem involving multiple levels and steps including genetics, surgical treatment, and auditory rehabilitation. the evaluation of NF2 disease should rely on the collaborative efforts of otolaryngology, neurosurgery, dermatology, ophthalmology, and hearing rehabilitation institutions. the treatment of NF2 lies not only in clinical surgery, but also in the selection of the timing of surgery, psychological treatment of the patient and family, and The treatment of NF2 is not only about clinical surgery, but also about the selection of the timing of surgery, the psychological treatment of the patient and family and the patient’s postoperative hearing rehabilitation.