Renal cell carcinoma (RCC) in children is relatively uncommon, especially in the absence of syndromes or hereditary diseases. Duplex kidney - as a common congenital anomalies in children - is usually insignificant with no serious impact or known risk of tumor association. Herein, we report a 5-year-old girl who presented with gross hematuria and a right renal mass in duplex system identified on computed tomography.
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