Diffuse involvement of the pulmonary interstitium with abnormal fibrous tissue is a process that occurs in many settings. There are many possible etiologies for pulmonary fibrosis, but in the majority of individuals, a clear cause cannot be determined and a diagnosis of idiopathic pulmonary fibrosis (IPF) results. Despite limited knowledge concerning the etiology, recent advances in biomedical technology offer great promise for increasing our understanding of IPF. This review will focus on current concepts of the pathogenesis and therapy of IPF.