As our understanding of the pathobiology and natural history of the various forms of interstitial lung disease (ILD) has evolved, so have our approaches to treating this heterogeneous group of lung disorders. The earliest pharmacologic agents used to treat various forms of ILD were corticosteroids, and corticosteroids are currently the mainstay of therapy for many forms of ILD. However, it has become clear that corticosteroids and other anti-inflammatory agents lack efficacy for many forms of ILD, such as idiopathic pulmonary fibrosis (IPF), and newer therapies that are in clinical trials target the fibrogenic process and/or secondary pulmonary hypertension (PH) that is present in various forms of fibrotic lung disease. Novel therapies, such as the use of biologic agents (antibodies and cell cycle inhibitors) or stem cell therapies will undoubtedly evolve as new research is performed and clinical trials are undertaken. Lung transplantation remains an option for advanced lung disease that is progressive and unresponsive to non-surgical therapies.