This randomized, double-blind, placebo-controlled parallel study, investigated the effect of regular aerobic training and postexercise protein-carbohydrate supplementation in patients with facioscapulohumeral muscular dystrophy (FSHD). Trained participants, demonstrated improved fitness, workload, and walking speed (10% [confidence interval (CI) 4%-15%], 18% [CI 10%-26%], 7% [CI 4%-11%], respectively, p < 0.001, number needed to treat = 2.1). Self-assessed physical capacity and health (Short Form-36) also improved. Muscle strength and daily activity levels did not change with training. Protein-carbohydrate supplementation did not result in further improvements in any tests compared to training alone. This study showed that regular endurance training improves fitness, walking speed, and self-assessed health in patients with FSHD without causing muscle damage. Postexercise protein-carbohydrate supplementation does not add any further improvement to training effects alone.