Idiopathic pulmonary fibrosis (IPF) is of unknown etiology, and the histopathology is mainly common interstitial pneumonia, which is confined to the lung of chronic fibrotic interstitial pneumonia. The disease has a high fatality rate and is harmful to human health. In recent years, with the aging of the population and the aggravation of air pollution, the incidence of IPF has increased year by year. Our understanding of IPF has also been improved. Studies have found that IPF patients are often complicated with other diseases, including emphysema, lung cancer and cardiovascular diseases. IPF patients with comorbidities had more clinical symptoms and poor quality of life. Among them, the combination of pulmonary hypertension can reduce the survival rate of IPF patients. This article reviews the combination of IPF and pulmonary hypertension in order to provide new ideas for the diagnosis and treatment of IPF and further research to improve the quality of life and prognosis of IPF patients.