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Granulomatosis with polyangiitis (GPA) is the systemic vasculitis affecting predominantly small vessels, but vasculitis of medium size artery can be associated. We treated a patient with GPA who had hemorrhagic instability because of a rupture of an aneurysm in the branch of the renal artery; the patient underwent arterial embolization (AE), and hemostasis was successfully achieved. Literature reviews were conducted on the basis of the data available on PubMed, and seven published reports of eight cases with renal artery aneurysms were