Birt-Hogg-Dubé syndrome (BHDS) (OMIM#135150) is a rare autosomal dominant tumor-predisposition syndrome caused by pathogenic variants in the FLCN gene encoding the folliculin protein which has been implicated in the regulation of cellular survival, proliferation, and growth by interacting with the mTOR signaling pathway [1, 2]. The gene discussed is FLCN; the disease is Birt-Hogg-Dubé syndrome.