Muscular dystrophy is a genetically diverse group of disorders that can affect both children and adults. Many forms of muscular dystrophy have significant cardiac manifestations. The most common cardiac consequences in muscular dystrophy are cardiomyopathy and arrhythmias. Correct identification of the genetic subtype of muscular dystrophy is critical for proper care since it determines the degree to which a cardiologist should be involved in the care of the patient. Cardiomyopathy and arrhythmias often develop later in the course of disease so cardiac management of the neuromuscular patient often falls within the remit of the adult cardiologist. However, since early treatment can be effective at slowing the progress of disease, prepathologic treatment and surveillance for arrhythmias may fall under the paediatric cardiologist. The myotonic dystrophies represent a subset of inherited muscular dystrophy disorders. Myotonic dystrophy, or dystrophia myotonica (DM), is a multisystem disease, and the cardiovascular defects may be disabling and life threatening. DM is dominantly inherited, so it is not uncommon that multiple family members of different generations are afflicted with the disorder. Cardiac involvement is prevalent in DM and accounts for approximately one third of the deaths in this population.1 2 This article discusses the recommendations for screening and risk stratification of cardiac complications in the DM population.