Birt-Hogg-Dube syndrome is characterized by cutaneous findings, including fibrofolliculomas, angiofibromas, fibroepithelial polyps, and trichodiscomas. It is an inherited autosomal dominant disorder. This syndrome also includes extra-cutaneous findings, such as pulmonary cysts, spontaneous pneumothorax, and renal cancer. Because of the systemic involvement, early diagnosis and treatment are important. Here, we report the case of a 53-year-old man diagnosed as having Birt-Hogg-Dube syndrome.