Introduction: Muscular dystrophies are a group of genetic muscular diseases with progressive degeneration of skeletal muscular fibres. The effects of exercise on the course of illness are not completely clear. Objectives: To review scientific evidence of the effects of exercise in patients with muscular dystrophy. Methods: Literature was obtained by using PubMed and Cochrane Library databases and web search engines Google Scholar and Cobiss. Search was limited to Slovenian and English language. The review was limited to randomized controlled trials only. Results: Nine studies met all the inclusion criteria of which four studies investigated effects of resistance training, four of aerobic training and one of a training programme combining both types of exercise. The results showed improved muscular strength and endurance with resistance training. The aerobic training was shown to enhance aerobic capacity, muscular strength and endurance, as well as keeping functional ability of the patient. The combined form of training positively influences vitality subscale of SF-36 questionnaire. No negative effects were reported. Conclusions: The type and volume of exercise training reported in reviewed studies is safe, feasible, and has modest positive effect on patients with different forms of muscular dystrophy.