Idiopathic pulmonary fibrosis (IPF) is a dreaded disease of uncertain etiology and no available cure. It is still unclear if a causal relationship exists between gastro-esophageal reflux (GER) and IPF, but studies have shown an increased prevalence of acid reflux in patients with IPF. We describe a patient with achalasia and GER who went on to develop IPF. She underwent a rapidly worsening course punctuated by acute exacerbations of IPF, despite best efforts to manage the acid GER. We also reviewed the literature on the role of GER in the etiology and progression of IPF and the impact of antireflux measures on its course.