TFEB/6p21/VEGFA-amplified renal cell carcinoma is a rare subtype of renal cell carcinoma that was first proposed as a separate subtype by Argani et al. in 2016 (ref. [1, 2]) and was not included in the WHO until 2022 due to its unique and rare nature. The gene discussed is TFEB; the disease is hereditary clear cell renal cell carcinoma.