Background: In many sports, maintaining balance is necessary to compete at a high level. Also, in many health problems, balance is impaired. Postural sway (PS) is often used as an indicator of upright balance control, and physical activity (PA) might enhance balance control. However, the relationship between PS and PA has never been systematically reviewed. Objective: Our objective was to summarize the evidence regarding the relationship between PS in upright bipedal and unipedal standing and PA. Methods: We conducted a literature search in MEDLINE, EmBase, CINAHL, the Cochrane Database, and PEDro, up to March 2012, with no limit on the starting date. Characteristics and methodological aspects of each article were extracted by two reviewers. We used centre of pressure (CoP) velocity, and variables related to the CoP area, to compare studies. Results: A total of 39 articles were reviewed from an initial yield of 2,058. Of these 39 studies, 37 used a comparative design, one was a cohort study, and one was a randomized controlled trial. Conclusion: The main conclusion was that in general, sport practitioners sway less than controls, and high-level athletes sway less than low-level athletes. Additionally, we identified specific effects dependent on the use of vision, sport-specific postures, and frequency and duration of the (sports) activity. PS in unperturbed bipedal stance appears to have limited sensitivity to detect subtle differences between groups of healthy people. © 2013 Springer International Publishing Switzerland.