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The paper presents the rare case of neurofibromatosis type 2 scheduled for hysterectomy. The 39 year old female patient admitted in obgy department with chief complaint of dysmenorrhea .Histrory od exaggeration of skin neurofibromas after first pregnancy moreover she was operated for vocal cord nodule one year back , presenting with CNS involvement .In this case report we are discussing the anesthetic challenges faced and the multidisciplinary approach required for successful management .