Behçet disease has been associated to pulmonary manifestations and may be lethal if not treated correctly. The authors describe a case of a 53 year-old male with aneurysmatic pulmonary artery dilatation, pulmonary hypertension, and hemoptysis. A review is presented about the diagnosis and the medical and surgical treatment of this disease.

Keywords: Behcet's syndrome. Pulmonary artery. Pulmonary hypertension. Aneurysm. Hemoptysis.