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Maltepe Üniversitesi, Tıp Fakültesi, Nöroşirürji Anabilim Dalı, İstanbul, Türkiye Neurofibromatosis Type 2 is an autosomal dominant disorder in which patients develop benign and malignant tumors at an increased frequency. Affected individuals typically present with bilateral schwannomas but meningiomas, ependymomas, astrocytomas, neurofibromas, and other cranial nerve and peripheral schwannomas are also commonly found. Although surgery remains the focus of current management, new, effective therapies are needed because of substantial morbidity and reduced life expectancy. Complexities associated with management of the multiple, progressive and protean lesions necessitate multidisciplinary treatment. Despite the significant progress toward a more complete understanding of the molecular basis for Neurofibromatosis Type 2, neither diagnostic criteria nor the strategies for management have reached a decisive point. In this study, we have reviewed the relevant literature to demonstrate the current knowledge in the pathogenesis, clinical findings and treatment modalities for Neurofibromatosis Type 2. Anahtar Kelimeler : Neurofibromatosis, Schwannomas, Pathogenesis, Treatment modalities