Idiopathic pulmonary fibrosis (IPF) is an incurable, debilitating disease which impairs lung function and eventually leads to death. Currently, there is a lack of effective modifying therapies and treatments for IPF as the underlying epidemiological mechanism is not clearly understood. This leads to difficulty in diagnosing and managing IPF, which results in a high incurment of disease-associated cost. Even though IPF poses a substantial economic burden, there is a lack of research available on cost triggers and healthcare utilization, which can be a barrier to future economic evaluations of new medicines for IPF. We aimed to conduct a systematic literature review (SLR) to identify the key cost-generating events of IPF and to gather any related costing information. The data showed that the main events triggering high resource use in patients were the symptoms of IPF progression along with comorbidities and lung transplantations. These events result in a high economic impact through the use of medications, health care professionals, and hospital stays. More research is needed to identify the direct, and indirect, relationships between IPF events and the costs they generate. This would help to further evaluate the area of need for future health technologies and to understand what events should be targeted to reduce the global economic burden of IPF.