It is now accepted that physical activity is not deleterious in myopathies, including muscular dystrophies. In patients suffering from facioscapulohumeral dystrophy (FSHD), aerobic training has been reported to be associated to physiological and functional positive effects without alteration in quality of life. The review papers from Van der Kooi et al. (2005), Cup et al. (2007) and Féasson et al. (2010) suggest that the combination of endurance and strength training is even more relevant. Only a few controlled and randomized studies have been conducted on this topic. The impact of such training programs on the skeletal muscle regenerative capacities has not been yet addressed. Moreover, due to the fact that training programs are mainly performed on short-term supervised periods, there is a lack of knowledge regarding long-term effects, patient's autonomy and whether or not regular exercise practice can be maintained in patient's daily life. Also, only a few experiments have reported an integrative view of the potential benefits of such programs on functional, biological and quality of life.