Birt-Hogg-Dube syndrome is characterized by clinical manifestations such as hamartomas of the skin, renal tumors and lung cysts with spontaneous pneumothoraces. Patients with Birt-Hogg-Dube syndrome may present with only multiple lung cysts. We report the chest computerized tomography (CT) features of three patients with BirtHogg- Dube syndrome. Each patient had multiple lung cysts of various sizes according to chest CT evaluation, most of which were located in lower lobes and related to pleura. The identification of unique characteristics in the chest CT of patients with Birt-Hogg-Dube syndrome may provide an efficient mechanism for diagnosis.