An 18-year-old man presented with Behçet's disease and two large bilateral pulmonary artery aneurysms. The day following aneurysmorrhaphy of the right aneurysm, a massive hemoptysis led to death. Postmortem examination disclosed rupture of the left aneurysm in a segmental bronchus. The aneurysms had inflammatory walls and were associated with organized pulmonary arterial thromboses and endomyocardial fibrosis of the right ventricle. Our case report highlights the surgical risk of rupture of associated arterial lesions, certainly related to per- and postoperative pulmonary arterial pressure variations.