Abstract: Objective: To analyze the clinical characteristics of antineutrophil cytoplasmic antibody （ANCA） associated vasculitis（AAV） in children and to improve its recognition. Methods: A retrospective analysis was performed for the clinical data of children with AAV who were hospitalized between March 1st,2010 and December 31st, 2019 in the Children's Hospital of Zhejiang University School of Medicine. Results: Among 10 AAV patients, there were 4 cases of granulomatosis with polyangiitis (GPA),4 cases of microscopic polyangiitis (MPA),and 2 cases of eosinophilic granulomatosis with polyangiitis (EGPA). Seven cases started from respiratory symptoms, 2 cases from renal lesions and 1 case from anemia. Cough, sputumdyspnea, hemoptysis, chest tightness or thoracalgia were the most common respiratory symptoms.The chest CT showed ground glass shadow in bilateral lungs, consolidation nodular opacities, honeycomb, bronchiectasis and cavity, pleural effusion, pleural thickening, emphysema and other manifestation. MPO or PR3 was positive in 8 cases and erythrocyte sedimentation rate elevated in all patients. Ten cases were treated with corticosteroid and 8 cases were combined with immunosuppressive agents. Clinical status improved in 9 cases and one children died. Conclusion: Respiratory involvement is the primary manifestation in children with AAV. The lung may be the only organ affected in the early stage of AAV. The respiratory manifestation is lack of specificity, which is easy to be misdiagnosed or missed. When the treatment of antibiotics is less effective in pneumonia patients with damage in multiple organs, it is suggested that further examination of ANCA and pathological biopsy should be conducted for early diagnosis.