BHD, caused by germline mutations in the tumor suppressor gene FLCN (encoding the protein folliculin, involved in cell growth), is associated with renal tumors of various histologic types in up to 19% of cases: hybrid oncocytic/chromophobe tumor, chromophobe tumor, oncocytoma, clear cell carcinoma, papillary carcinoma, and mixed-type carcinoma. Here, FLCN is linked to papillary carcinoma.