A 59-year-old man showed asynchronous development of noninfectious granulomatous aortitis with aneurysm, followed 9 months later by pulmonary capillaritis and glomerulonephritis in association with elevated serum antineutrophil cytoplasmic antibody levels. To our knowledge, the combination of large artery vasculitis and small vessel systemic vasculitis (capillaritis) has not been previously reported. This case may represent a hybrid type of systemic vasculitis combining features of giant cell arteritis and Wegener's granulomatosis.