Renal carcinoma associated with Xp11.2 translocations and TFE3 fusions (Xp 11.2 RCC) form a new and little known entity of the WHO classification. It accounts for at least one-third of pediatric RCCs and 15% of RCCs in patients <45 years of age. Renal carcinoma associated with Xp11.2 translocations and TFE3 fusions (Xp 11.2 RCC) is a rare pediatric renal carcinoma diagnosed by fluorescence in situ hybridization (FISH). We report a case of Xp 11.2 RCC in a 13 year old girl, presented with hematuria. Mass was detected on CECT. She underwent nephrectomy and histopathological examination confirmed renal cell carcinoma. Type was confirmed by FISH. Renal cell carcinoma in pediatric age with characteristic morphology need to be subcategorized using ancillary methods. The most sensitive and specific immunohistochemical markers for these neoplasms are TFE3 protein and cathepsin-K3. Complete surgical removal of the tumour mass including the kidney is the preferred therapy in patients with lower stage ...........