[Lethal pulmonary involvement in neurofibromatosis]

A unusual case of a 61-year-old man who presented dyspnea and cough associated to skin and subcutaneous lesions typical of neurofibromatosis type 1 is reported. Chest roentgenograms and CT-scan showed emphysematous bullae. The patient underwent drainage of the greatest bulla, but death eventually ensured as a consequence of upper digestive bleeding. It can be expected that with the longer survival of neurofibromatosis patients with respiratory troubles, that are generally considered unusual will be recognized more frequently.