The four main syndromes von Hippel–Lindau disease (VHL, caused by pathogenic variants in VHL); hereditary leiomyomatosis and renal cell cancer (HLRCC, caused by pathogenic variants in FH); Birt-Hogg-Dubé syndrome (BHD, caused by pathogenic variants in FLCN); and hereditary papillary renal carcinoma (HPRC, caused by pathogenic variants in MET) are considered to be responsible for hereditary RCC subtypes [4]. This evidence concerns the gene FH and von Hippel-Lindau disease.