Lung transplantation has been available as a therapeutic option for patients with end-stage cystic fibrosis lung disease for over 15 years, but the outcome following transplantation remains poor, and the supply of organs limited. For this reason some children opt to continue with medical treatment followed by terminal care rather than undergo transplantation. This article summarizes the current status of lung transplantation, including current referral guidelines and contraindications and also addresses current practice in terminal care.