Although TS and BHD are clearly unique syndromes, recent articles demonstrated that both FLCN protein (mutated in BHD syndrome) and tuberous sclerosis complex (TSC) protein (mutated in TS syndrome) lead to activation of mammalian target of rapamycin complex 1 (mTORC1) of the mTOR pathway. This evidence concerns the gene MTOR and Birt-Hogg-Dubé syndrome.