BackgroundProficiency in gross motor skills (GMS) lays the foundation for developing more complex motor skills. Improving these motor skills may provide enhanced opportunities for the development of a variety of perceptual, social, and cognitive skills. However, GMS development and intervention effects are not ideal for many non-typically developing children.ObjectiveTo systematically evaluate the effect of active video games on the development of gross motor skills in non-typically developing children and adolescents.MethodsSeven Chinese and English databases were searched for randomized controlled trials, and the risk of bias in included studies were qualitative evaluation according to the revised Cochrane risk of bias tool for randomised trials (RoB 2). Then a meta-analysis was conducted to estimate the overall effect of active video games on the development of gross motor skills in non-typically developing children.ResultsTwenty papers were included. In the three subordinate concepts of gross motor skills, active video games significantly improved locomotor skills [ SMD = 0.59, 95% CI (0.40, 0.77)] and non-locomotor skills [SMD = 0.51, 95% CI (0.20, 0.81)] in non-typically developing children. However, there was no significant difference compared with the control group [ SMD = 0.32, 95% CI (− 0.17, 0.82)] in object control skills.ConclusionsThe study shows that active video games can improve locomotor skill and stability skill in non-typically developing children, but the effect on object control skill is uncertain, and more high-quality literature needs to be included in the future.Trial registration The meta-analysis was registered on INPLASY (202,250,124) and is available in full on inplasy.com (https://inplasy.com/inplasy-2022-5-0124/).