This review focuses on vasculitides with prominent pulmonary manifestations and discusses key contributions from the recent literature. Pulmonary vasculitis should be considered when clinical findings include alveolar hemorrhage, nodular and cavitary lung disease, airway stenosis, pulmonary artery aneurysms, or pulmonary artery stenosis. The differential diagnostic considerations for common clinical presentations of vasculitis in the lung are important, and several recent additions are discussed. Treatment for established pulmonary vasculitis is effective and has decreased the morbidity and mortality associated with these diseases while introducing an increased risk of infectious complications. Advances in immunosuppressive therapy have improved treatment of refractory disease and are likely to change initial treatment strategies in the future.