Lung transplantation (LTx) is an established therapeutic option for patients with various end-stage lung diseases. Presently the worldwide procedural frequency is ∼ 3,200 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 for LTx are emphysema, pulmonary fibrosis, and cystic fibrosis. Worldwide, a recent analysis of all recipients reported that 23% had idiopathic pulmonary fibrosis (IPF) and 3% had pulmonary artery hypertension. In experienced centers, candidates for transplantation are chosen according to disease-specific factors after the exclusion of contraindications. 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. Nevertheless, LTx offers a survival benefit in carefully selected patients with interstitial lung diseases and pulmonary hypertension.