Patients with mutations in the BHD gene develop renal cell carcinomas (RCC), the majority of which are chromophobe and chromophobe/oncocytic hybrid tumors, and somatic BHD mutations have been found in sporadic chromophobe RCC, which is a rare disease affecting ∼1500 individuals in the United States each year. Here, FLCN is linked to hereditary clear cell renal cell carcinoma.