ObjectiveTo investigate the diagnosis and treatment of neurofibromatosis（NF）firstlydiagnosed in otolaryngology head and neck surgery.MethodsＴhe clinical data of 23 patients with NF which was received from 1990 February to 2014 May were retrospectively analyzed.The clinical presentation、diagnosis and management were discussed combined with literatures review.Results8 cases were conducted preoperative pathology, 20 cases were neurofibroma by postoperative pathology diagnosis.After followup for 28 months (2 months~10 years）, 4 cases were dead, 1 case died of systemic metastasis of neurofibromas with malignant schwannoma, 1 case which was bilateral acoustic neuroma died of brain stem hemorrhage after the second operation， the other 2 cases died of neurofibroma with malignant transformation and systemic metastasis；19 cases were still alive，among them, 3 cases occurred recurrence, including 1 case malignant transformation.ConclusionSuperficial skin type multiple neurofibroma just only need observation with regular followup.Larger tumor in otolaryngology head and neck or solitary neurofibroma which has local compression symptoms or impact function and hairdressing, should be conducted surgical resection.If malignant transformation appears, wide excision assisted with postoperative standard radiotherapy and chemotherapy is a better choice.