Congenital renal arteriovenous malformation is a rare disease. Renal arteriovenous malformations have been discovered with increasing frequency since they were first described by Varela in 1923. Patients with arteriovenous malformations may have gross hematuria, flank pain, urinary obstruction or may be asymptomatic. The diagnosis of arteriovenous malformation is usually made by renal angiogram. Treatment of arteriovenous malformation can be obtained with conservative treatment, ligation of the arterial branch, selective transcatheter embolization and surgical procedures. We experienced a case of congenital arteriovenous malformation manifested by gross hematuria and intermittent right flank pain, cured by means of transcatheter arterial embolization therapy with gelfoam and alcohol. Thus, we report a case with a brief review of literatures.