EoRCC is in some cases linked to pathogenic germline variants (PGVs) in genes associated with RCC familial syndromes (VHL, MET, FLCN, TSC1, TSC2, FH, SDHx, PTEN, BAP1) [1–3]; these genes are also often somatically mutated in sporadic RCC cases [2–5]. The gene discussed is VHL; the disease is renal cell carcinoma.