We have previously reported the presence of germline deletions in two other familial forms of kidney cancer, Birt–Hogg–Dubé (BHD), caused by germline mutations in the folliculin (FLCN) gene, and hereditary leiomyomatosis and renal cell carcinoma (HLRCC), resulting from germline fumarate hydratase (FH) gene mutations (Benhammou et al., 2011; Vocke et al., 2017). The gene discussed is FLCN; the disease is hereditary clear cell renal cell carcinoma.