OBJECTIVES: We investigated renal outcome of kidney-transplantation in Korean recipients with biopsy-proven renal involvement of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in a single centre. METHODS: We reviewed the medical records of 144 Korean patients and included 3 female patients with microscopic polyangiitis (MPA) and one male patient with eosinophilic granulomatosis with polyangiitis (EGPA) in this study. We obtained clinical and laboratory data related to kidney-transplantation, analysed renal outcome of kidney-transplantation in 4 recipients with AAV and compared it with those of previous studies. RESULTS: The mean age at diagnosis was 37.8 years and that at kidney-transplantation was 40.8 years. Time-gap from AAV to ESRD ranged from 1 to 48 months and that from AAV to kidney-transplantation ranged from 2 to 95 months. All kidney-recipients with had been followed-up for two years or greater. At diagnosis, MPO-ANCA was detected in only MPA patients, while, at transplantation, MPA-ANCA was detected in 2 MPA patients and an EGPA patient. All patients have received tacrolimus and mycophenolate mofetil based on glucocorticoid after kidney-transplantation. Among 4 kidney-recipients with AAV, one MPA patient underwent renal re-biopsies three times due to renal function deterioration during the follow-up. Renal histology revealed T cell-mediated and antibody-mediated rejection rather than relapse of MPA. Neither relapse nor graft failure was observed in our study. CONCLUSIONS: Renal outcome of kidney-transplantation in recipients with AAV was good and kidney-transplantation is deserved to be recommend as a safe and effective therapeutic modality to AAV patients with ESRD.