A 56-year-old female patient with MPO-ANCA and anti-GBM double-seropositive ANCA-associated vasculitis (AAV) experienced progressive renal damage, complex pulmonary infection and refractory severe anemia. During immunosuppressive and anti-infective treatments, high-dose erythropoiesis-stimulating agent (ESA) with intermittent blood transfusion had no significant effect on her anemia and hemoglobin level fluctuated at 57–67 g/L. Roxadustat, an oral HIF-PHI, was initiated a standard dose of 100 mg three times weekly, along with monthly cyclophosphamide immunosuppressive therapy, leading to hemoglobin level increasing steadily and reaching to 134 g/L after 3 months, and stable at approximately 120 g/L with the dose of 50 mg three times weekly. With the improvement of anemia, the primary autoimmune disease was alleviated significantly, manifested in the remission of hematuria and hemoptysis, the decrease in the serum MPO-ANCA and anti-GBM levels, and the reduction in lung lesions. The combination of severe anemia and ANCA-associated renal vasculitis often result in poor long-term renal prognosis and a shortened lifespan. This case demonstrates roxadustat is less strongly affected by the inflammatory state of the body and thus can treat renal anemia in patients with ANCA-associated renal vasculitis safely and effectively, which provides valuable experience in the treatment of anemia in patients with immune kidney diseases.