A case of combined anomalies of the urinary tract, horseshoe kidney, retrocaval ureter and pelviureteric stenosis, is presented. Routine urological examination of a three-year-old girl who complained of recurrent cystitis, showed horseshoe kidney and right hydronephrosis. Surgical exploration revealed the retrocaval ureter, and the isthmus of the horseshoe kidney lying between the aorta and the inferior vena cava. The pelviureteric junction itself was also stenotic and compressed from behind by the aberrant vessels. In addition to the dismembered pyeloplasty in front of the vena cava, isthmotomy was performed. The literatures on such combined anomalies were reviewed.
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