Arteriovenous malformations of the kidney with renal artery aneurysm are rare. Rupture of an congenital arteriovenous malformation in the kidney has been reported only in 4 cases and all cases were accompanied with pregnancy. We present a case of spontaneously ruptured congenital arteriovenous malformation of the kidney with renal artery aneurysm in a 39-year-old man with literature review.