A 67-year-old woman, who presented with a 2 month history of dyspnea, had a ventilation and perfusion lung scan that showed absent perfusion of the entire right lung with normal ventilation, as well as a rounded matched defect in the left lower lung adjacent to midline, suspicious for an aortic aneurysm or dissection. CT pulmonary angiography revealed a massive descending aortic aneurysm compressing the right pulmonary artery as well as the left lung parenchyma, accounting for the bilateral perfusion scan defects. We present the Xe-133 ventilation, Tc-99m MAA perfusion and CT pulmonary angiography imaging findings of this rare case.