Primary ciliary dyskinesia (PCD) impairs pulmonary function, respiratory and peripheral muscle strength, and exercise capacity. We aimed to investigate the effects of active video games (AVGs) on pulmonary function, respiratory and peripheral muscle strength, exercise capacity, muscle oxygenation (SMO2), physical activity, activities of daily living (ADL), and quality of life (QOL) in PCD. Thirty-two PCD patients were randomly assigned to AVG group (n =16) and the control group (n=16). AVG group underwent AVGs using Xbox-Kinect-360 device for 40 min/day, 3 days/week for 8 weeks plus airway clearance techniques (ACT), and the control group was applied ACT only. Pulmonary function, respiratory and quadriceps muscle strength, exercise capacity (6-min walk test [6MWT], incremental shuttle walk test [ISWT]), and ADL (Glittre ADL test) were assessed. SMO2 during ISWT and ADL test was also recorded. Physical activity and QOL (PCD-QOL) were evaluated. Pulmonary function; respiratory and quadriceps muscle strength; 6MWT and ISWT distance; physical activity; ADL performance; SMO2; physical, emotional, and social functioning; treatment burden; and upper and lower symptom parameters of PCD-QOL significantly improved after 8 weeks in the AVG group (p < 0.05). There were no significant differences in measured parameters except emotional function and upper respiratory symptom scores of PCD-QOL in the control group (p > 0.05).