Congenital renal arteriovenous malformations are rare, but their incidence are frequency of recognition are rising. Six cases of both cirsoid and idiopathic varieties are described. Patients were either asymptomatic or presented with gross hematuria and flank pain. Physical findings included hypertension, cardiomegaly, flank tenderness, and an abdominal bruit. IVP findings included filling defects in the renal pelvis from blood clots, irregular mucosal pattersn, or mass effect. Angiography demonstrated either single, simple, vascular channels or multiple, complex, varix-like communications. In both there was early filling of the renal vein, a normal caliber to the feeding artery and draining vein, and no displacement of parenchymal vessles. Asymptomatic patients required no treatment. Surgical procedures that spare renal parenchyma are preferred.