Among benign tracheobronchial neoplasms, neurofibromas of neurogenic origin are exceedingly rare. In a search world literature, only 23 cases of endotracheobronchial neurofibromas in 21 series were found. We report another case of a 52-year-old man who presented to our hospital with the symptoms of intermittent productive cough and fever. Bronchoscopy revealed a round tumor obstructing the lumen of the right main bronchus. Right sleeve pneumonectomy was performed, and neurofibroma was confirmed by pathologic examination. The literature is reviewed.