The first case of left retrocaval ureter connected with situs inversus and unilateral renal agenesis and, in addition, one case of retrocaval ureter in normal situs are reported. In the first case, the renal pelvis was transsected, the retrocaval part of the ureter was dissected free and resected. The continuity was re-established by ureter-pelviplasty ventral of the vena cava inferior. In the second case only poor clinical manifestations were estimated, there was no pyelectasis in the IVP. Surgical treatment was not necessary at the moment. Embryology, clinical aspects, diagnosis and the different surgical procedures are discussed.

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