History and clinical findings | A 57-year-old woman with a history of emphysematous COPD GOLD D and homozygotic alpha-1-antitrypsin deficiency presented with aggravated dyspnoea, presyncope and left thoracic chest pain. 12 years previously Stanford type B dissection had been conservatively treated. Examinations | Chest radiograph revealed extended infiltrates of the left lower lobe. Seen together with elevated inflammatory values and the patient´s hemodynamic instability it pointed at a pneumonia-driven septic shock. In view of a progressive respiratory insufficiency and an acute drop in haemoglobin computed tomography was performed. It showed rupture of an aneurysmatic left lower pulmonary artery with consecutive intraparenchymal bleeding. Treatment and course | Because of persistent bleeding and respiratory failure requiring invasive ventilation, thoracotomy was performed. Left lower pulmonary artery was sutured and left lower lobe resected. After initial postoperative stabilization bronchopleural fistula demanded re-thoracotomy and parenchymal lesions of left upper lobe were sutured. In consequence of progressive sepsis and manifest multiorgan failure patient died 15 days after initial surgical bleeding control. Conclusion | Though homozygotic alpha-1-antitrypsin deficiency is primarily associated with lung and liver disease, extrapulmonary and extrahepatic manifestation may occur. In view of consistent case reports and plausible pathogenetic mechanisms, vascular complications comprising arterial aneurysms and dissection due to compromised vascular integrity in alpha-1-antitrypsin deficient individuals is conceivable, but lacks scientific proof.