Background: An increasing body of evidence has suggested that idiopathic pulmonary fibrosis is inclined to combine with lung cancer in recent years. Thus we studied the correlation of the two diseases to raise awareness of the risk of lung cancer in pulmonary fibrosis patients among doctors and patients. Methods: Data about the epidemiology, pathogenesis, clinical features and treatment strategies of idiopathic pulmonary fibrosis associated lung cancer cited in this review were obtained from major databases such as Pubmed, Web of Science, Embase, Scoups, Cancerlit and so on from 1957 to 2015. Results: The prevalence rate of lung cancer in pulmonary fibrosis patients is much higher than that in general population. Perturbed signaling pathways, epigenetic and genetic changes, and oxidative stress are all thought to be involved in the pathogenesis of idiopathic pulmonary fibrosis associated lung cancer. Hemoptysis and chest pain should be an alert for the presence of lung cancer in pulmonary fibrosis patients. The peripheral, lower, and highly fibrotic regions are the most common sites of lung cancer. Adenocarcinoma and squamous cell carcinoma are the most common types in idiopathic pulmonary fibrosis associated lung cancer patients. The treatment strategies including medication and surgery are controversial, complex and thorny. Conclusion: Idiopathic pulmonary fibrosis is associated with an increased lung cancer risk. It is necessary to raise awareness of lung cancer risk in idiopathic pulmonary fibrosis patients. Further research investigating the pathogenesis of idiopathic pulmonary fibrosis associated lung cancer could help develop newer therapeutic targets for treatment of idiopathic pulmonary fibrosis and lung cancer.