Retrocaval ureter presenting at 6 years of age in a girl child – An extreme rarity

Retrocaval ureter is one of the very rare congenital anomalies. We report a case of retrocaval ureter in a 6-year-old girl who presented with right flank pain and hydronephrosis. The diagnosis was made on intravenous urography which showed typical “J” shape deformity in the proximal dilated ureter with moderate hydronephrosis. CT scan delineated the course of ureter. The patient was operated and findings were confirmed. The ureter was transected near the pelvis and a pyeloureteric anastomosis with pre-caval transposition of the ureter was performed and the patient was discharged in fair health.