Mucoviscidosis is a genetic disease caused by the malfunction of Cystic Fibrosis Transmembrane Regulator. This disease is manifested primarily in the respiratory and gastrointestinal systems. Cystic fibrosis affects skeletal system, leads to low bone mineral density and causes osteopenia and osteoporosis. Bone density changes in cystic fibrosis depends on disorders of the respiratory and gastrointestinal systems or are associated with genetic alterations. The most frequently used bone density test is densitometry, which is performed every two to five years. Determining the level of vitamins D, K and calcium and bone turnover markers. Treatment of cystic fibrosis is consisting of pharmacotherapy and physiotherapy. Pharmacological treatment is primarily supplementation vitamins D, K and calcium as well as bisphosphonates, parathyroid hormone and growth hormone therapy. Unfortunately pharmacotherapy of mucoviscidosis is difficult due to mutual interaction of drugs and negative effect of certain drugs e.g. steroids on bone density. Pharmacotherapy treatment should be used together with physiotherapy. Essential rehabilitation elements are respiratory therapy, whole body exercises and physical activity in everyday life. These activities affect the long-therm improvement in treatment effects and leads to increasing of lifetime and quality of life of the patients. Consequences of cystic fibrosis e.g musculoskeletal pain, posture defects and diabetes are more frequently occurs and require using appropriate strategy of physiotherapy.