Cystic fibrosis is a hereditary progressive disease caused by a mutation of the CFTR gene. It produces complications in several organ systems; the most affected being the respiratory system, towards which physiotherapy interventions are directed. They include airway clearing techniques with an emphasis on drainage positions with percussion and vibration and kinesitherapy. They enable patients to expel inflammatory secretions from the respiratory tract, easier and more efficient expectoration, strengthening of the respiratory muscles, improvement of respiratory function which eases facilitating daily activities. Before determining adequate interventions, it is necessary to make an individual assessment of the respiratory system and functional abilities. In the assessment, pulmonary function tests and stress tests are distinguished. The treatment of cystic fibrosis still focuses on the symptoms, however, with the advancement of medicine, more and more efforts are being made to treat and act on the cause of the disease, which will prolong the life span of the affected. High expectations are placed on medication known as CFTR modulators, as they have already shown certain positive effects in recent trials, but a more detailed analysis is needed for the use of these drugs as standard therapy.