Objective: To conduct a systematic review to evaluate the effectiveness of Pilates intervention on physical function in children and youth. Data sources: Six electronic databases were searched from inception to June 2018 using the term Pilates. Study selection: Articles were included if they (1) reported original data for a Pilates-only intervention; (2) involved children or youth aged up to 22 years; (3) reported a musculoskeletal, pain, or function study outcome. Searches identified 2565 papers and 11 studies fulfilled the inclusion criteria. Data extraction: The 2 authors independently screened and assessed all studies and any discrepancies were resolved by consensus. Data synthesis: Level of evidence was classified using the Oxford Centre for Evidence Based Medicine. Study quality was assessed using the Physiotherapy Evidence Database for randomized controlled trials and Risk of Bias in N-of-1 Trials scale for single case experimental design studies. Four studies were high quality, 3 were fair quality, and 4 were of low quality. Findings showed that Pilates does appear to improve flexibility (n=6); muscle strength, power and movement speed (n=3); postural control, orientation and balance (n=3); metabolic cost (n=1); functional ability (n=1) and health related quality of life (n=1) and reduce pain (n=2) in children with musculoskeletal pathology, the majority with a large effect size. Pilates content varied from group-based mat classes to individualized programs using specialized equipment. Intervention dose and frequency varied widely. Conclusions: This is the first systematic review of the effect of Pilates intervention for children and youth. Research is in the preliminary stages; however, Pilates does appear to improve flexibility, strength and postural control, and reduce pain in children with musculoskeletal pathology. Further research is warranted to determine the potential effectiveness of Pilates for children and youth for various population groups and to develop comprehensive treatment guidelines.