Combined pulmonary fibrosis and emphysema (CPFE) is a syndrome characterized by the presence of emphysema in the upper lobes of the lungs and fibrosis in the lower lobes. Diagnosis of CPFE is challenging because emphysema and fibrosis have opposing effects on lung function, which may mask typical symptoms and imaging findings. CPFE patients have a poorer prognosis and higher mortality compared to patients with only chronic obstructive pulmonary disease (COPD) or idiopathic pulmonary fibrosis (IPF). Worsened survival is associated with the extent of fibrosis and emphysema, older age, and complications such as lung cancer, acute exacerbations, and pulmonary arterial hypertension. The pathogenesis of CPFE involves common mechanisms such as transforming growth factor beta (TGF-β), which affects lung tissue healing and fibrogenesis. CPFE is diagnosed based on clinical symptoms, lung function, and imaging methods, especially CT scans. The management of the disease does not yet have uniform standards and requires a multidisciplinary approach. This review summarizes knowledge of CPFE from 1990 to 2024, including epidemiology, clinical presentation, diagnosis, course, and treatment[1][6][8].