Retrocaval ureter is a rare malformation occasionally associated with other urological or nonurological abnormalities. We report an exceptional case of retrocaval ureter associated with contralateral pelvic ectopic kidney in a 22 year old man. The anomalies associated with the retrocaval ureter and the embryological mechanisms are described. Abdominal computed tomography with 3D reconstruction provided a new perspective of this malformation, but its clinical value remains unclear.