Right Ventricular Dysplasia constitutes a genetic cardiomyopathy characterized by fibrous-adipose substitution of the right and rarely of the left ventricular myocardium. This disorder is associated with ventricular arrhythmias ranging from frequent ventricular ectopic beats, nonsustained and sustained ventricular tachycardia of left bundle branch morphology and sudden death. Therefore, the syndrome has been labelled Arrhythmogenic RVD Cardiomyopathy. Diagnostic criteria, preliminary genetic data, and clinical manifestations are summarized and critical addressed, using data from the literature and from our own experience. The most important aspects of the ECG in this syndrome are reviewed and stressed with particular attention to initial versus advanced clinical subsets. The typical anatomical abnormalities and biopsy or pathology material are presented.