We encountered an extremely rare case of a renal arteriovenous fistula (AVF) with 2 giant renal artery aneurysms (RAAs) in a 75-year-old woman who presented with congestive heart failure and pulmonary hypertension. A chest x-ray revealed cardiomegaly with a cardiothoracic ratio (CTR) of 65%. Computed tomography showed 2 giant left RAAs (diameter, 45 × 40 mm2 and 75 × 60 mm2) associated with an AVF, dilated inferior vena cava, and dilated pulmonary arteries. A radical nephrectomy was performed through a transverse laparotomy after the left renal vein, and arteries were dissected from the inferior vena cava and aorta, respectively. The patient’s postoperative course was uneventful, and her condition improved. Her cardiomegaly was ameliorated, as manifested by a decrease in CTR to 57% and the absence of volume overload.