The muscular dystrophies represent an array of inherited myogenic disorders involving mutations and dysregulations of a variety of cytoskeleton or nuclear proteins. Many muscular dystrophy patients also develop a concomitant cardiomyopathy. Due to a greater understanding of the underlying pathogenesis of many of the muscular dystrophies and advances in neurological and pulmonary care, the primary mode of death today in many of these patients is cardiovascular in nature. However, despite the high incidence of cardiomyopathy in many muscular dystrophy patients there is limited data regarding optimal management of their underlying cardiomyopathy. Therefore, the objective of this Medical Grand Rounds is to enhance ones understanding of the assessment and management of neuromuscular-associated cardiomyopathy.