Background: Joint hypermobility syndrome (JHS) is a heritable connective tissue disorder characterised by excessive range of movement at multiple joints accompanied by pain. Exercise is the mainstay of management yet its effectiveness is unclear. Objectives: To establish the effectiveness of therapeutic exercise for JHS. Design: Systematic literature review. Data sources: A search of nine online databases, supplemented by a hand search and snowballing. Study eligibility criteria (participants and interventions): People diagnosed with JHS (rather than asymptomatic generalised joint laxity); therapeutic exercise (of any type) used as an intervention; primary data reported; English language; published research. Study appraisal and synthesis methods: Methodological quality was appraised by each reviewer using Critical Appraisal Skills Programme checklists. Articles were then discussed collectively and disagreements resolved through debate. Results: 2001 titles were identified. Four articles met the inclusion criteria, comprising one controlled trial, one comparative trial and two cohort studies. All studies found clinical improvements over time. However there was no convincing evidence that exercise was better than control or that joint-specific and generalised exercise differed in effectiveness. Limitations: The studies used heterogeneous outcome measures, preventing pooling of results. Only one study was a true controlled trial which failed to report between-group statistical analyses post-treatment. Conclusions and implications of key findings: There is some evidence that people with JHS improve with exercise but there is no convincing evidence for specific types of exercise or that exercise is better than control. Further high quality research is required to establish the effectiveness of exercise for JHS. © 2013 Chartered Society of Physiotherapy.