Background Youths with Down syndrome are characterized by deficits in balance/postural stability. One way to palliate balance deficits among this population is through exercise interventions. However, to the authors’ knowledge, the effects of exercise interventions designed to improve the balance of youths with Down syndrome have never been systematically reviewed. Purpose The purpose of this review was to summarize the findings from studies examining the effects of exercise interventions designed to improve balance in youths with Down syndrome. Data Sources A systematic literature search was performed in 10 databases (Academic Search Complete, CINAHL Plus With Full-Text, Education Source, ERIC, Medline With Full-Text, PsycARTICLES, Psychology and Behavioral Sciences Collection, Scopus, SocINDEX, and SPORTDiscus With Full-Text) on June 12, 2017. Study Selection Randomized controlled trials and controlled trials examining the effects of exercise interventions designed to improve balance in youths with Down syndrome were included. Data Extraction Two authors selected the studies and extracted their characteristics and results. Three authors assessed the risk of bias in the studies using the Cochrane Collaboration tool. Data Synthesis Eleven studies, published between 2010 and 2017, met the inclusion criteria. The findings showed that exercise interventions were more effective than control conditions for improving the static balance of children with Down syndrome and the static-dynamic balance (ie, global balance score obtained with a scale measuring both static and dynamic balance) of children and adolescents with Down syndrome. Nevertheless, the findings on dynamic balance in children and static balance in adolescents were inconclusive. Limitations With a small number of studies and their high risk of bias, the present findings must be interpreted with caution. Conclusions The reviewed exercise interventions were successful in improving the static balance of children with Down syndrome and the static-dynamic balance of children and adolescents with Down syndrome.