Objective To investigate the clinical features of anti-neutrophil cytoplasmic antibody(ANCA)-associated vasculitis and to analyze the causes of misdiagnosis.Methods The clinical data of 3 patients with misdiagnosed ANCA-associated vasculitis as pulmonary tuberculosis admitted from July 2021 to July 2022 were retrospectively analyzed.Results One patient presented with cough,hemoptysis,and shortness of breath,one with hemoptysis,and one with cough and dyspnea.All three patients had lung involvement and were misdiagnosed as pulmonary tuberculosis,and the misdiagnosis las-ted 4-29 months.ANCA-associated vasculitis was confirmed by ANCA profile.One patient was relieved after treatment with glucocorticoids,and 2 patients did not use glucocorticoids,of whom one patient was lost to follow-up and one died after deteri-oration.Conclusion The clinical manifestations of ANCA-associated vasculitis lack specificity,and clinicians should be alert to the disease and improve ANCA profile testing as soon as possible to reduce missed diagnosis and misdiagnosis.