In the last 2 decades, diagnosis of congenital renal arteriovenous malformation has increased owing to the availability of angiography. Recently, color Doppler ultrasound has played an adjunctive role in the detection of renal A VM for its non-invasive means and efficiency. Meanwhile, the treatment of choice has changed from nephrectomy to transarterial embolization for minimizing renal parenchymal damage. In this report, congenital renal AVM leading to gross hematuria was diagnosed in 3 females. Three lesions were all detected by color Doppler ultrasound and were proved by renal angiography. Superselective transarterial embolization was performed in these 3 cases. For 1.5-yr follow-up, no more microscopic or gross hematuria was found.