Abstract: Objective　To explore the clinical features, diagnosis and treatment of Anti-neutrophil cytoplasmic antibody associated vasculitis (ANCA) in children, and raise awareness of the disease. Methods　The clinical data of 3 children with ANCA associated vasculitis were retrospectively analyzed from 2013 to 2017 in the hospital. Results　Of the 3 cases , 2 were girls and 1 boys . One case was positive for MPO, one case was positive for P-ANCA, one case was negative for P-ANCA after one year of treatment, and the other two cases were negative for ANCA related antibodies because of pulmonary infection. The initial performance of 3 cases was different. 2 cases had different degrees of renal injury, 2 cases had different degrees of lung injury, 1 case had skin involvement, and 1 case had liver involvement. In case 2 , after the therapy of glucocorticoid combined with cyclophosphamide, the patient had improved significantly. However, the patient was automatically discharged from hospital due to recurrence of infection and economic problems during the course of the disease. Cases 1 and 3 were treated with hormones, support, etc., and their condition improved and discharged. Conclusions　The first clinical manifestation of ANCArelated vasculitis in children vary widely, and when multiple organs such as kidney and lungs are damaged , we should be alert to ANCA-related vasculitis , and early diagnosis and early treatment can improve their prognosis .