Background: Researchers of epidemiological studies in recent years have witnessed the expansion of children with neurodevelopment disorders (NDDs), particularly of children with attention-deficit/hyperactivity disorder (ADHD), autism spectrum disorder (ASD), Down syndrome (DS), developmental coordination disorder (DCD), Cerebral palsy (CP). Noticeable motor deficits are among common characteristics of children with NDDs, which indicate the need for interventions to promote optimal motor and overall development. While physical activity (PA) has been widely used in the rehabilitation of children with NDDs to improve their motor performance, questions remain whether or not the beneficial effects of PA are conclusive. The purpose of this study was to conduct a systematical review and metal analysis of the studies investigating the effects of PA on motor skill performance in children with NDDs. Methods: Relevant articles were sourced from PubMed, the Web of Science, EBSCO, the Cochrane Library, CNKI and Wanfang data. Results: Twenty studies with 269 subjects met inclusion criteria for this review and were included. Compared with pre-interventions, there was a significantly improve in motor skills(Fig.1 & 2), the gross motor skills (SMD [standardized mean difference] = 0.64, 95%CI [coefficient interval]: 0.29 to 0.99), fine motor skills (SMD = 1.33, 95%CI: 0.57 to 2.09) respectively. Conclusion: PA could effectively improve gross and fine motor skill performance in children with NDDs. Although the findings were based on a small number of studies, the results of this meta-analysis still suggest that researchers and clinicians should consider including PA in their rehabilitation programs for children with NDDs. Future studies should clarify which type and what intensity of PA intervention might be most effective in promoting motor development based on individual children’s needs and treatment characteristics.