Aims : To describe current criteria for the early and late diagnosis of cystic fibrosis (CF) and cystic fibrosis transmembrane conductance regulator (CFTR) related disorders; to describe methods of preventing, diagnosing and treating the most common respiratory infections in CF; to describe the importance of regular exercise testing, daily activity and physiotherapy in the long-term treatment of CF; to describe the long-term treatment of patients with stable CF, including the treatment of frequent co-morbidities; and to describe the criteria for treating patients with CFRT modulators.