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To update readers on current recommendations for timing of lung transplantation in individuals with end-stage cystic fibrosis lung disease and on the rationale behind listing decisions.Guidelines for the referral and selection of patients suitable for lung transplantation were recently updated by the pulmonary council of the International Society for Heart and Lung Transplantation. However, an analysis published in 2007 has questioned whether lung transplantation extends life in children with cystic fibrosis. There are some concerns regarding this analysis, and these are discussed in detail. Most importantly, the analysis is specific to the United States and predates the introduction of the lung allocation score, which has had a marked impact on how transplant organs are allocated in this country.It is likely that lung transplantation can extend life in both adults and children with cystic fibrosis, provided the procedure is correctly timed. Further development of the lung allocation score has the potential to increase the survival benefit from the procedure in the United States.