Arrhythmogenic right ventricular dysplasia is a hereditary, progressive and unpredictable disease of the heart muscle that is often diagnosed after a patient's sudden cardiac death. The disease is classified as a cardiomyopathy. It affects men more often than women, and the incidence of symptoms is highest in young adulthood. Pathohistologically, it is the programmed death of cardiac muscle cells that are replaced by fibrous-fatty tissue. The disease most often affects the right ventricle, but it can also start in the left ventricle and bilaterally. Genetic desmosomal mutations are inherited mainly in an autosomal dominant manner and are associated with the development of the disease. The clinical presentation includes syncope, palpitations, arrhythmias and sudden cardiac death. In the diagnosis of the disease, the Task Force diagnostic criteria are used, which include 2D echocardiography, cardiovascular MR, right-sided ventriculography, electrocardiogram, biopsy with histological analysis of the heart muscle and family history with genetic testing. It is also necessary to consider the differential diagnosis, especially cardiac sarcoidosis, myocarditis and Burgada syndrome, which resemble arrhythmogenic right ventricular dysplasia in their clinical picture. Treating the disease is a big challenge. It is crucial to modify the lifestyle in the form of a ban on physical exertion, as well as other therapeutic methods, depending on the clinical findings. Catheter ablation, pharmacotherapy and implantation of an implantable cardioverter defibrillator are used for primary or secondary prevention of sudden cardiac death. Translational treatment methods are the future in patients with arrhythmogenic right ventricular dysplasia, but much more research is needed for it to enter clinical practice.