We report on a 45-year old male who underwent evaluation for lung-transplantation because of end-stage chronic obstructive pulmonary disease due to homozygous alpha-1-antitrypsin-deficiency. Routine coronary angiography at that time was normal, however, the patient did not consent to the operation then and two years later all examinations were repeated, when the clinical condition had worsened. Repeated angiography now revealed a severe ectatic coronary vasculopathy. We hypothesize that the simultaneous effect of elastine-degraduation in arterial walls by uninhibited activity of proteolytic elastase and constant hypoxemia due to the patient´s progressive lung disease caused the rapid development of coronary aneurysms.