Abstract: Pulmonary fibrosis (PF) is a chronic, progressive, fibrotic lung disease that causes irreversible decline in lung function, progressive respiratory failure, and even death. The pathogenesis of PF has not been fully elucidated. Current studies have shown that pulmonary fibrosis is characterized by repetitive microscopic alveolar epithelial cell injury and dysregulated repair, fibroblast proliferation and excessive deposition of extracellular matrix (ECM), resulting in loss of parenchymal architecture and fibrosis. In recent years, with increased understanding of PF pathogenesis, two therapeutic drugs (pirfenidone and nintedanib) have been approved for marketing worldwide, and more than ten are under clinical decelopment. This paper reviewed the pathogenesis associated with pulmonary fibrosis and the progress of clinical researches on the latest therapeutic drugs.