Pulmonary involvement in patients with neurofibromatosis has been repetitively reported as a very rare complication in type 1 variety. It is characterized by pulmonary intersticial disease, pulmonary fibrosis and bullaes, the last with high risk of rupture. We described a case of spontaneous pneumothorax in a patient with type 2 neurofibromatosis, as consequence of pulmonary fibrotic changes. To our knowledge this association had not been reported.
