Combined pulmonary fibrosis and emphysema (CPFE) is a syndrome which is usually seen in male smokers and characterized by severe dyspnea, preserved lung volume and significant deterioration in diffusion capacity. Radiological characteristics are upper-lobe emphysema and lower-lobe fibrosis. Etiopathogenesis is unclear but in genetically predisposing patient’s environmental factors such as smoking are thought to be the major causes. Pulmonary hypertension is frequent complication and has a prognostic value. In the treatment emphysema and fibrosis should be managed separately, and great effort must be taken to prevent disease progression and complications. Lung transplantation must be considered in suitable patient population.