<p>Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is characterized by small vessel inflammation, which can lead to renal hemorrhage. Some forms of ANCA-associated vasculitis, such as microscopic polyangiitis, may lead to aneurysm formation, particularly renal aneurysm, which may resolve with appropriate immunosuppressive therapy. However, maintenance treatment should be administered cautiously to patients with ANCA-associated vasculitis and end-stage renal disease (ESRD) owing to the lower relapse rate and increased risk of infectious complications associated with immunosuppressive therapy. Herein, we describe a case involving a woman with ANCA-associated vasculitis and ESRD who did not receive maintenance immunosuppressive therapy and subsequently experienced right and left renal hemorrhages 8 and 14&#xa0;years after the initiation of dialysis, respectively. Treatment choices for ANCA-associated vasculitis are based on individual factors such as disease severity and level of vasculitis activity. This case report highlights ANCA-associated vasculitis with two episodes of aneurysm-related bleeding, emphasizing that maintenance immunosuppressive therapy may be critical for patients having ANCA-associated vasculitis with ESRD, particularly those with persistently high ANCA titers.</p>