Myopathies are genetic or acquired disorders of skeletal muscle that lead to varying degrees of weakness, atrophy, and exercise intolerance. In theory, creatine supplementation could have a number of beneficial effects that could enhance function in myopathy patients, including muscle mass, strength and endurance enhancement, lower calcium levels, anti-oxidant effects, and reduced apoptosis. Patients with muscular dystrophy respond to several months of creatine monohydrate supplementation (~0.075-0.1 g/kg/day) with greater strength (~9%) and fat-free mass (~0.63 kg). Patients with myotonic dystrophy do not show as consistent an effect, possibly due to creatine transport issues. Creatine monohydrate supplementation shows modest benefits only at lower doses and possibly negative effects (cramping) at higher doses in McArdle's disease patients. Patients with MELAS syndrome show some evidence of benefit from creatine supplementation in exercise capacity, with the effects in patients with CPEO being less robust, again, possibly due to limited muscle creatine uptake. The evidence for side effects or negative impact upon serological metrics from creatine supplementation in all groups of myopathy patients is almost non-existent and pale in comparison to the very substantial and well-known side effects from our current chemotherapeutic interventions for some myopathies (i.e., corticosteroids).