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Background  Congenital giant megaureter (CGM) is uncommon in the pediatric population. The major clinical presentations are marked protruberances and abdominal cysts.    Case presentation  We reported a case of CGM with almost the whole left ureter dilation accompanied with a 1 cm stricture at the entrance of the bladder and renal dysplasia, immediately compressing the contralateral ureter and causing bilateral hydronephrosis for the first time. At one-stage of the operation, a left nephrostomy with a right ureterolysis were performed, and a poor left kidney function was found. Then, the left kidney and ureter were cut off by nephroureterectomy at the second-stage. Eventually, the follow-up showed that the patient recovered well by abdominal ultrasound.    Conclusion  Based on the findings of these reported literatures, CGM is rare. The physical and imaging examinations are essential for the diagnosis of CGM, and the appropriate treatment methods should be performed based on patients’ specific condition.

bmc urology

Unilateral congenital giant megaureter with renal dysplasia compressing contralateral ureter and causing bilateral hydronephrosis: a case report and literature review