Birt-Hogg-Dubé (BHD) is a familial syndrome that leads to the development of lung cysts and carries a significantly increased risk of spontaneous pneumothorax. Other manifestations are benign hair follicle tumors and a high incidence of renal cancers. The diagnosis is often first considered when unexplained lung cysts are found. Recognition of the syndrome by chest physicians is important because of the risk of spontaneous pneumothorax and because BHD patients and families require screening for renal cancers. Clinical features of BHD are given as well as recommendations for management of patients with BHD and their families.