In patients with syncope, ventricular tachyarrhythmias, and sudden death, in the absence of coronary artery disease or dilative and hypertrophic cardiomyopathy, arrhythmogenic right ventricular disease is a common underlying heart disease. It is marked by ventricular tachyarrhythmias of left bundle branch block pattern, often induced by exercise; negative T-waves in the right precordial leads of the surface-ECG, and localized or diffuse right ventricular contraction abnormalities detected by various imaging techniques in a young, apparently healthy population. Congenital disposition with progressive fibrous and lipomatous tissue infiltration of the right ventricular myocardium with hypertrophy and degeneration of surviving myocytes is the pathogenetic factor leading to modification of conduction and resulting in the development of arrhythmogenic zones. This review contains the current knowledge about arrhythmogenic right ventricular disease on the basis of our own experience, and also a review of the literature. Controversial hypotheses concerning etiology and prognosis are discussed. Variant forms and stages of the disease are described, taking differential diagnostic aspects into consideration. Non-invasive and invasive diagnostic methods are evaluated with regard to their diagnostic accuracy, feasibility, and limitations. Pharmacological and alternative therapeutic concepts are presented, including catheter ablation and surgical intervention.