The underlying disease of a candidate for lung transplantation, especially advanced pulmonary fibrosis, can cause particular and dramatic difficulties. Pulmonary fibrosis is the end-result of a variety of pathological diseases and their associated processes. This article summarizes the diagnosis and management of some of the more common causes of fibrosis, outlines their natural histories and treatment outcomes, and describes the trade-off of pulmonary fibrosis for lung transplantation. Four main categories of end-stage fibrosis are discussed: idiopathic pulmonary fibrosis, sarcoidosis, pulmonary fibrosis from systemic diseases or drugs, and occupational- or environmental-related pulmonary fibrosis. Each group will be covered systematically and the options and indications for lung transplantation will be addressed.