We report a rare case of right retrocaval ureter and left nutcracker syndrome in a young man who was accurately diagnosis preoperatively with the help of intravenous urogram and gray-scale and color flow Doppler ultrasonography, and confirmed by computed tomography. Surgery that involves resection of the retrocaval segment and relocation of the anastomosis anterior to the inferior vena cave was performed for the right retrocaval ureter, whereas surveillance was adopted for the left nutcracker syndrome.