Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are a necrotizing autoimmune vasculitis that occurs as a result of immune-mediated damage to small and medium-sized vessels. Three primary clinicopathological syndromes were defined in the AAV; Granulomatosis with Polyangiitis (GPA), Microscopic Polyangiitis (MPA) and Eosinophilic Granulomatosis with Polyangiitis (EGPA). The disease may manifest itself as alveolar hemorrhage due to systemic inflammatory response, purpuric rash due to vascular rupture, or segmental glomerular infarction due to vascular occlusion. The lungs are the organs commonly affected in AAV. Lung involvement in AAV can be listed in 5 main categories. These are pulmonary capillaritis characterized by granulomatous inflammation (lung nodules), tracheobronchial inflammation, diffuse alveolar hemorrhage (DAH), interstitial lung disease (ILD) and asthma. DAH and ILD are associated with poor prognosis. The aim of treatment is to achieve remission and prevent relapses.