Renal cell carcinoma in a duplex kidney in pediatric

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. Radical nephrectomy was performed; the patient was diagnosed with Xp11 translocation RCC and no other modalities of treatments were needed over 3-month follow-up.