Neurogenic tumours arising in the trachea are rare; the association with Von Recklinghausen's disease is exceptional. Only two cases of tracheal neurofibroma with neurofibromatosis have been reported. We report on another patient, a thirty year old man, who presented with acute shortness of breath; laser resection was performed via an endotracheal approach, then, a few days later, resection of the exotracheal tumor and tracheal segment was performed. Postoperatively, the patient did well for nine years without any respiratory complaint or neurofibromatosis relapse.