Abstract: Xp11 translocation renal cell carcinoma is a rare renal cell carcinoma subtype harboring a TFE3 translocation, which is grouped into the MiT family translocation renal cell carcinomas together with t (6;11) translocation renal cell carcinomas. With the development and application of immunohistochemistry, fluorescence in situ hybridization, reverse transcription-polymerase chain reaction, and RNA sequencing, increasingly more Xp11 translocation renal cell carcinomas have been diagnosed and studied, and many new insights have been elucidated and advances have been made in clinicopathology, molecular genetics, and clinical treatment. The latest progress in Xp11 translocation renal cell carcinoma research is introduced and reviewed in this paper.