The present review gives an overview of the clinical and subclinical manifestations of cardiac involvement (CI) in Becker muscular dystrophy (BMD), its pathophysiological background, diagnostic possibilities and therapeutic options for CI in BMD patients and carriers. CI may be subclinical or symptomatic. Up to 100% of patients develop subclinical CI. The onset of symptomatic CI is usually in the third decade of life, rarely in the first decade. One-third of patients develop dilative cardiomyopathy with concomitant heart failure. In BMD patients, CI manifests as electrocardiographic abnormalities, hypertrophic cardiomyopathy, dilation of the cardiac cavities with preserved systolic function, dilative cardiomyopathy or cardiac arrest. There is no correlation between CI and the severity of myopathy. CI is more prominent in patients than carriers. As soon as the diagnosis of BMD is established, a comprehensive cardiac examination should be performed. Because CI in BMD is progressive and adequate therapy is available, cardiac investigations need to be regularly repeated. If CI in BMD is recognized early, appropriate therapy may be applied early, resulting in a more favourable outcome.