Neurofibromatosis is wide spread hamartoblastomasis of various connective, nervous and supporting tissue of the body and its autosomal dominant inheritance is well known. The disease is characterized by multiple tumor of skin, cutaneous pigmentation and neurofibroma of peripheral and central nervous system. In 1860 Kölliker described some aspect of neurofibromatosis for the first time but later the clinical entity was established by Von Recklinghousen in 1882. The interesting feature of this disease to otolaryngologist is not only the combined bone anomaly in skull and vertebrae but also its frequent association with VIII nerve tumor and occational occurrence in the soft tissue of the neck, oral cavity, pharynx, larynx, trachea and mediastinum. Our department has experienced 11 cases of neurofibromatosis with co-relational trouble in ENT field for last 8 years and detail analysis of history, clinical finding and difficulty on surgical management is presented with review of literature.