Cystic fibrosis (CF) is the most common, life-threatening, and recessively inherited disease in the Western world. This chapter provides an overview of the condition, including its aetiology, pathology, and genetics. It then covers the clinical presentations and confirmation of diagnosis of CF, complications (respiratory, gastrointestinal, pancreatic, bowel, hepatic, reproductive, and diabetic). Anyone recognized as having high risk of having a baby with CF and in whom pregnancy is contemplated, or who has a newborn with CF, should be offered screening and early genetic advice. Care and management are described in detail, including nutrition, physiotherapy, infection control, antibiotics, the transition from paediatric to adult care, transplant, and end-of-life care.