Autosomal dominant neoplastic syndrome characterised by genodermatosis, lung cysts, spontaneous and recurrent PNEUMOTHORAX; and RENAL CANCER. It is associated with mutations in the folliculin protein gene (FLCN protein).

Synonyms: Hornstein-Birt-Hogg-Dubé Syndrome, Hornstein-Knickenberg Syndrome, Birt-Hogg-Dube Syndrome, Birt Hogg Dube Syndrome, Fibrofolliculomas with Trichodiscomas and Acrochordons, Birt-Hogg-Dubé Syndrome