Diffuse tracheobronchial neurofibromatosis is a rare condition, and its clinical manifestations include obstruction, cough, wheezing, and dyspnea. Furthermore limited data make treatment decisions challenging. In addition airway papillomatosis tends to affect the upper airway and the larynx in the form of well-delimited lesions leading to obstruction, predominantly in children. Diffuse involvement of the trachea and the bronchial tree and its association with neurofibromatosis have been rarely reported in adults. We present a patient diagnosed with neurofibromatosis of the trachea complicated by papillomatosis.