Abstract: Idiopathic pulmonary fibrosis is a kind of two pulmonary fibrosis disease characterized with honeycomb change, its incidence and mortality rate increased year by year, and lifetime is short, the poor prognosis is almost the same with malignant tumor. Therefore, pathogenesis and treatment of idiopathic pulmonary fibrosis has become the focus in the international medical community. The paper reviewed the disease mechanism and drug treatment research progress, to provide a reference for the further research.