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Translocation renal cell carcinoma (t-RCC) is a subtype of renal cell carcinoma (RCC). This case is about an 11-year-old with pain in right iliac fossa for two weeks. Tumor of 2.2 x 1.9 x 2.8 cm was evidenced in the upper pole of the right kidney, by ultrasound. Then, she underwent to a right radical nephrectomy and regional lymph node dissection, after which CRT subtype Xp 11.2 was diagnosed. Subsequently, first-line treatment with pazopanib and concomitant chemotherapy was initiated. This disease is more frequent between 10 and 15 years. Their prognosis is usually better compared to adults. The multidisciplinary management associated with the availability of first-line medications improves progression-free survival.