For selected parenchymal lung disease patients who fail to respond to medical therapy and demonstrate declines in function that place them at increased risk for mortality, lung transplantation should be considered. Lung transplantation remains a complex medical intervention that requires a dedicated recipient and medical team. Despite the challenges, lung transplantation affords appropriate patients a reasonable chance at increased survival and improved quality of life. Lung transplantation remains an appropriate therapeutic option for selected patients with parenchymal lung disease.