Annotation. This study is devoted to evaluation of clinical course of cystic fibrosis (CF) in children. The study involved 84 children who were diagnosed with cystic fibrosis. All participants were asked about complaints, life history, physical examination was made, and the results of objective testing and instrumental examination were assessed. The statistical system “IBM SPSS Statistics” version 12 (20) was used using parametric and non-parametric methods of descriptive statistics. Student's criterion was used to assess the significance of the difference between the independent samples, and the Fisher's factor was used for the percentage data. It was shown that 86.91% of subjects had severe or moderate course of CF. Major mutation of CFRT gene F508del/F508del was found in 45.2% children. Common complaints were productive cough (92.9%; р<0.01), failure to thrive (61.9%; р<0.01) and shortness of breath during exercise (40.5%; р<0.01). Results of bacteriologic test of sputum showed significant prevalence of St. aureus (54.0%; р<0.01) and Ps. aeruginosa (38.10%; р<0.01). Patients with severe course of CF had lower rates of FVC, FEV1, PEF, FEF 25%, FEF 50%, FEF 75% than children with moderate and mild course. At the age of 3 years, the diagnosis of cystic fibrosis was established in time by only 76.92% of respondents, which aims to further efforts to improve the quality of its diagnosis.