OBJECTIVES: We investigated the incidence rate and type of cancer, and furthermore, estimated the standardised incidence ratios (SIRs) of cancer in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in Korea. METHODS: We retrospectively included 150 patients with AAV [81 patients with microscopic polyangiitis (MPA), 38 with granulomatosis with polyangiitis (GPA) and 31 patients with eosinophilic GPA (EGPA)], and reviewed their medical records. We collected demographic, clinical and laboratory data, and reviewed the use of glucocorticoid and immunosuppressive drugs administered until detection of cancer or last visit. We estimated the SIRs of cancer according to totality, gender, age, AAV variants and each type of cancer. RESULTS: The mean age at diagnosis of AAV was 55.2 years and that at last visit was 59.5 years. The mean follow-up duration was 50.7 months. Four of 150 AAV patients had cancer (2.7%), and they got four different types of cancers including gastric cancer, lung cancer, prostate cancer and Non-Hodgkin lymphoma. Two patients exhibited gap-time from AAV to malignancy less than one year, and the rest of them exhibited gap-time of 8 and 6 years, respectively. The overall SIR of cancer in AAV patients was 1.43 (95% confidence interval 0.391, 3.671). The SIRs of cancer based on gender, and age at cancer or last visit, AAV variants and each type of cancer were not significant, either. CONCLUSIONS: The risk of cancer is low in Korean patients with AAV.