The majority of hereditary RCCs are caused by pathogenic germline variants in the VHL gene (OMIM #608537) that causes von Hippel-Lindau syndrome (VHL), while other predisposing syndromes include hereditary leiomyomatosis and RCC (FH, OMIM#136850), Birt-Hogg-Dubé (FLCN, OMIM #607273), hereditary papillary RCC (MET, OMIM#164860), hereditary paraganglioma and RCC (SDHB, OMIM#185470) and constitutional chromosome 3 translocations of t(3;8)(p14.2;q24.1) (reviewed in [8], OMIM#14470). Here, VHL is linked to renal cell carcinoma.