Functional Electrical Stimulation (FES) is a new rehabili- tative approach and refers to the process of pairing the electri- cal stimulation with a functional task, as cycling or walking, in persons unable to perform actively these movements. In the last years, the important role of FES is emerging in neurological diseases with severe muscle weakness, as stroke or spinal cord injury. The role of FES in neuromuscular disorders has never be evaluated. The aim of the study was to evaluate the effects of FES in improving lower limbs muscle strength, endurance and gait speed in Myotonic Dystrophy type 1 (DM1). Five DM1 patients were enrolled in the study. Three patients performed FES training while the other two carried out strength exercises. The modified Medical Research Council (MRC) scale, the Six Minutes Walking test (6MWT), the time to cover 10 meters (10mWT) and muscle MRI were used for the assessment at the baseline and at the end of the treatment. A statistically significant improvement of muscle strength (p = 0.0008) and of the 6MWT (p = 0.02), emerged only in those patients who performed FES training. No significant changes in gait speed was observed. Muscle MRI showed a reduction of intramuscular fatty infiltration after FES training. This study suggests that, in DM1, FES can be considered a valid and safe method to improve endurance and muscle strength, even in those muscle with severe weakness in which no other re- habilitative options are otherwise available. It also highlights the need to perform future controlled trials in this field.