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Background The SARS-CoV-2 pandemic has become the most serious health problem of today globally. Kidney involvement in patients with coronavirus disease 2019 (COVID-19) is common and associated with high mortality. Although acute tubular necrosis due to hemodynamic instability is the most common cause, other complex and destructive processes related to cytokine storm and activation of innate and adaptive immunity have also been reported. Case presentation: Herein, we present successful treatment of proteinase-3 anti-neutrophil cytoplasmic antibodies (PR3-ANCA)-associated vasculitis case presenting with severe pulmonary-renal syndrome as a rare and fatal complication of COVID-19 infection. Conclusions AAV is a serious disease and prompt treatment is one of the most important factors in patient survival.