Muscular dystrophies comprise an heterogeneous group of diseases characterised by primary wasting of skeletal muscle, in the most severe forms leading to progressive paralysis and death. Current therapies for these conditions are extremely limited and based on corticosteroids that bear significant side effects. Several studies have proposed possible alternative strategies, ranging from cell and gene therapy to more classical pharmacological approaches. Nitric oxide is a gaseous messenger involved in many mechanisms responsible for preserving muscle function and stimulating muscle repair. We herein review the most recent pre-clinical and clinical findings that open new prospective for the development of nitric oxide as a therapeutic tool for muscular dystrophies.