Right ventricular dysplasia is a primary disorder of the right ventricular myocardium characterised by a progressive replacement by adipose or fibrous tissue, proceeding from the epicardium towards the endocardium. Right ventricular dysplasia with the predominant clinical manifestation of an arrhythmia of right ventricular origin is defined as arrhythmogenic right ventricular dysplasia. There is, however, a wide spectrum of clinical presentation and physical findings owing to the polymorphism of the condition. The aetiology of right ventricular dysplasia remains unknown. Right ventricular angiography is currently regarded as the gold standard for the clinical diagnosis of right ventricular dysplasia Ventricular endomyocardial biopsy can be useful in confirming the diagnosis but a negative biopsy does not exclude the diagnosis of right ventricular dysplasia. There have been few electrophysiological studies and these, in general, have failed to help in risk stratification. Treatment is focused on the prevention of potentially lethal right ventricular electrical instability.