Cystic fibrosis occurs in 1 in 2000 children and the majority now reac
h adulthood. The disease is a complex multisystem disorder which is li
kely to challenge anaesthetists with increasing frequency. In this rev
iew the presentation and genetics of the disease are briefly described
, followed by a detailed account of the pathophysiology relevant to an
aesthesia. The pre-operative assessment and conduct of anaesthesia are
discussed and some suggestions made regarding management.