Cystic fibrosis is genetic disease caused by malfunction in the cystic fibrosis transmembrane regulator. It is characterized by abnormally thick, sticky mucus which primary have formations in the lungs, digestive tract and reproductive track. Formation of thick, sticky mucus in the lungs create suitable ground for developing of infections which cause furthered degradation of lungs tissue, and in many cases the outcome of disease depends on condition of the respiratory disease. Cystic fibrosis needs to be recognized much earlier, and after that came transdisciplinary treatment aimed at achieving better quality and length of life of patient with cystic fibrosis. Important role in treatment of cystic fibrosis have physiotherapy. Process of physiotherapy involves a thorough assessment which helps in setting goals and choosing physiotherapy procedures, and physiotherapy interventions which is consisted of the application of these procedures. This review emphasizes and summarizes physiotherapeutic procedures whose common goal is to promote mobilization and elimination of mucus from the respiratory tract, improve ventilation and vital capacity, and enhance the right motions of lungs during breathing. The task of physiotherapist is to choose a technique that will achieve this quickly and efficiently, taking into account the characteristics and abilities of patient at the moment of therapy. Generally, physiotherapeutic intervention involves the airway clearance therapy, techniques of breathing and various forms of physical activity. Timely, early, and daily intervention can delay progression of respiratory disease and contribute to long-term survival of cystic fibrosis.