Objectives: Cystic Fibrosis (CF) is a chronic, progressive and life-shortening disease. Life expectancy has increased substantially in recent decades due to early diagnosis and improved treatment. Health-related quality of life (HRQOL) measures are patient-centered and provide information about the patient’s own assessment of his respiratory symptoms, physical, social, and emotional functioning and wellbeing. Methods: 40 children with CF completed the age-appropriate Russian version of the CFQ-R. Forced expiratory volume in 1 second (FEV1% predicted), body mass index (BMI) and intravenous antibiotic treatment (IV) were utilized as measures of health status. Conclusion: On average, scaled scores on the CFQ-R were as follows: Physical Functioning (70.9±25.5), Emotional Functioning (72.2±15.6), Eating Disturbances (69.7±25.7), Treatment Burden (74.4±18), Social Functioning (63.7±17.7), Body Image (82.7±21.6), Respiratory Symptoms (67.7±25.7) and Digestive Symptoms (72.5±24.9). Intraclass correlation coefficients measuring parent–child agreement ranged from r = 0.47 to 0.74 (p’s <0.001), indicating very good agreement. Significant associations were also found between lung function and CFQ-R Physical Functioning (r = 0.36, p = 0.02) and Respiratory Symptoms (r = 0.55, p = 0.000). No associations were found between BMI and CFQ-R scales. A trend was also found between number of IV courses and CFQ-R Physical Functioning (r = −0.43, but p = 0.07). This pilot study of the Russian version of the CFQ-R demonstrated strong convergent validity. This Russian translation of the CFQ-R can be used to evaluate HRQOL in children with CF, including use in drug trials and observational studies.