Cystic fibrosis is an inherited disease affecting mainly the Caucasian population. In recent years there have been major advances in understanding the genetics and pathophysiology of the disease. Cystic fibrosis affects many organs, but predominantly the respiratory system and alimentary tract. Current treatment including chest physiotherapy, antibiotics, good nutrition and enzyme therapy, together with the complications of the disease are described. Even with the best modern treatment there is still a significant morbidity and mortality. New advances in treatment, which offer hope for the future, are explored.