An overview over the role of lung transplantation in interstitial lung diseases will be given.Lung transplantation is an established therapy option for patients with various end-stage lung diseases. Currently, the worldwide procedural frequency is about 3600 per year. Unfortunately, the shortage of donor organs leads to approximately every sixth patient in western countries dying before a donor organ is available. The most frequent underlying clinical indications are emphysema, pulmonary fibrosis, and cystic fibrosis. In a recent registry report worldwide, 23% of all lung transplant recipients had a diagnosis of idiopathic pulmonary fibrosis. In experienced centers, candidates for transplantation are chosen according to disease-specific factors after excluding contra-indications. However, there are several challenges for lung transplantation. The number of lung transplantations performed is limited by the supply of donor organs, and the long-term survival rates are still inferior compared with other forms of solid organ transplantation.Lung transplantation offers a survival benefit in carefully selected patients with interstitial lung diseases.