The HLA-B27-related spondyloarthopathies are associated with cardiovascular disease in 2% to 10% of cases. Inflammation and sclerosis of the aortic root and ventricular septum have been linked to the development of isolated aortic regurgitation and conduction abnormalities; however, aneurysms of the aortic sinuses and coronary arteries have not been previously described. We report the case of a 58-year-old white man who presented for evaluation of dyspnea and was found to have aneurysms of the sinuses of Valsalva and the circumflex coronary artery. The patient underwent aortic root replacement. Approximately 3 months later, he presented with symptoms, radiographs, and laboratory data consistent with ankylosing spondylitis. To our knowledge, these particular cardiovascular manifestations of HLA-B27-related disease have not been previously reported. This case expands the clinical spectrum of the disease and should prompt the clinician to consider the possibility of HLA-B27-associated cardiovascular disease in patients who have aortic and coronary aneurysms.