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Renal cell carcinoma is the most common malignant renal tumour in adults but quite rare in children. Duplex renal collecting systems are relatively common congenital renal abnormalities. However, its coexistence with renal cell carcinoma is yet to be established beyond a mere coincidence. So far, there are only 2 cases of such coexistence in literature till date. We therefore report a case of a 13-year-old girl who was referred to us with a right flank mass. Abdominal computerized tomographic scan revealed a right renal tumour necessitating a laparotomy at which a duplex collecting system was discovered in the ipsilateral kidney and a nephrouterectomy was done with a histologic finding of renal cell carcinoma. We report this rare relationship and review of literature to stimulate further study into this rare association.

Renal cell carcinoma with ipsilateral duplex system: Case report and review of literature