Xp11.2 RCC is a rare subtype of RCC which results from gene fusions between the transcription factor E3 (TFE3) gene and at least 5 fusion partners including ASPL-TFE3, PRCC-TFE3, PSF-TFE3, CLTC-TFE3, and Nono -TFE3, whose chromosomal rearrangement is t(X;17)(p11.2;q25), t(X;1)(p11.2;q21), t(X;1)(p11.2;p34), t(X;17)(p11.2;q23) and inv(X)(p11.2;q12), respectively [8, 24]. The gene discussed is TFE3; the disease is renal cell carcinoma.