Abstract: Idiopathic pulmonary fibrosis (IPF) is a chronic progressive lung disease. It is one of the most common forms of idiopathic interstitial pneumonia with high mortality rate. The median survival time from diagnosis is only 2 to 3 years. Though the pathogenesis of IPF is still unclear, it has been found that some signaling pathways play important roles in the occurrence and development of IPF. Currently, pirfenidone and nintedanib are the main drugs approved by FDA for the treatment of IPF, and more than 10 drugs are in clinical studies. This article reviews the research progress of IPF related signaling pathways and the drugs in clinical development.