Therefore, the combination of the AR inhibitor MDV3100 and the UCHL1 inhibitor 6RK73 was effective in delaying the progression of Xp11.2 tRCC, especially PRCC‐TFE3 tRCC. Here, PRCC is linked to renal cell carcinoma associated with Xp11.2 translocations/TFE3 gene fusions.