Congenital arteriovenous fistula(AVF) of the kidney is a rare condition. We report a case of renal AVF with chief complaints of macroscopic hematuria, and treated with transcatheter arterial embolization (TAE) using coils. The patient was diagnosed as having left renal AVF by Color Doppler ultrasound. The signs and symptoms disappeared after embolization. The clinical course of a case is reported and the literature is reviewed. Color Doppler ultrasound is required to give a definite diagnosis of renal AVF.