Muscular dystrophy (MD) connotes a heterogeneous group of inherited disorders characterized by progressive wasting and weakness of the skeletal muscles. In several forms of MD, cardiac dysfunction occurs, and cardiac disease may even be the predominant manifestation of the underlying genetic myopathy. Cardiologists may be unfamiliar with these diseases owing to their low incidence; also, significant advances in respiratory care have only recently unmasked cardiomyopathy as a significant cause of death in MD. 1 Early detection of MD-associated cardiomyopathy is important, because institution of cardioprotective medical therapies may slow adverse cardiac remodeling and attenuate heart failure symptoms in these patients. 2–6 Although ECG and echocardiography are typically advocated for screening, 7, 8 cardiovascular magnetic resonance (CMR) has shown promise in revealing early cardiac involvement when standard cardiac evaluation is unremarkable. 9, 10 This review will focus on 4 groups of skeletal muscle disease most commonly associated with cardiac complications (the Table):(1) dystrophin-associated diseases such as Duchenne and Becker (DMD and BMD, respectively),(2) Emery-Dreifuss MD (EDMD),(3) limb-girdle MD (LGMD), and (4) myotonic dystrophy (DM).