Renal cancer is a prevalent urologic malignancy, with an annual incidence of 0.43 million and an annual mortality of 0.15 million.[1]TFE3‐rearranged renal cell carcinoma (TFE3‐RCC) is a highly aggressive subtype with limited treatment options in advanced stages, resulting in poor prognosis.[2, 3, 4, 5] Moreover, TFE3‐RCC is often misdiagnosed as kidney renal clear cell carcinoma (KIRC) or kidney renal papillary cell carcinoma (KIRP).[6, 7] because of a lack of reliable diagnostic markers.[8, 9] Therefore, accurate diagnostic methods and therapeutic targets for TFE3‐RCC are urgently needed. This evidence concerns the gene TFE3 and hereditary clear cell renal cell carcinoma.