Xp11.2 translocation renal cell carcinoma (Xp11.2 tRCC), also known as TFE3-fusion associated RCC, was listed as a new entity in the 2004 WHO classification of renal tumors and was recently included in the MiT (microphthalmia transcription factor) family tRCC as a new subset of RCC in the 2016 WHO classification [1, 2]. The gene discussed is TFE3; the disease is renal cell carcinoma.