Transcription factor E3 (TFE3) oncofusions are frequently detected in the Microphthalmia transcription factor (MiT) family translocation renal cell carcinoma (tRCC), a rare pediatric renal cancer with limited treatment options. This evidence concerns the gene TFE3 and renal cell carcinoma associated with Xp11.2 translocations/TFE3 gene fusions.