The lungs are affected by systemic vasculitides with differing frequency, characteristics, and severity, depending on the specific vasculitis syndrome. Alveolar hemorrhage, nodules, interstitial disease and airway stenoses are the most common lung manifestations of antineutrophil cytoplasm antibody-associated vasculitides. Pulmonary vascular involvement also occurs in Behçet disease and Takayasu arteritis, where it manifests as pulmonary artery aneurysm, thrombosis and stenoses. Lung involvement usually occurs along with other systemic features of the vasculitis syndrome but can also be isolated leading to diagnostic and therapeutic challenges. Lung infection, pulmonary edema due to cardiac failure or renal impairment, cancer, and drug-induced lung toxicity are potential mimickers of pulmonary involvement in vasculitis. The management of lung disease in patients with vasculitis includes systemic and local therapies.