The presence of pulmonary vasculitis can be suggested by a clinical presentation that includes diffuse pulmonary hemorrhage, acute glomerulonephritis, chronic refractory sinusitis or rhinorrhea, imaging findings of nodules or cavities, mononeuritis multiplex, multisystemic disease, and palpable purpura. Serologic tests, including the use of cytoplasmic antineutrophil cytoplasmic antibody (ANCA) and perinuclear ANCA, are performed for the differential diagnosis of the diseases. A positive cytoplasmic ANCA test result is specific enough to make a diagnosis of ANCA-associated granulomatous vasculitis if the clinical features are typical. Perinuclear ANCA positivity raises the possibility of Churg-Strauss syndrome or microscopic polyangiitis. Imaging findings of pulmonary vasculitis are diverse and often poorly specific. The use of a pattern-based approach to the imaging findings may help narrow the differential diagnosis of various pulmonary vasculitides. Integration of clinical, laboratory, and imaging findings is mandatory for making a reasonably specific diagnosis.