Arrhythmogenic right ventricular dysplasia (ARVD) is a genetic disease associated with sudden cardiac death, affecting subjects in the 3rd and 4th decade of life. Despite great efforts made in order to improve its early diagnosis, ARVD remains as a major public health problem in Europe and America. Currently, risk stratification of sudden cardiac death in patients with ARVD remains challenging. Over the last decade implantable defibrillators have been shown to increase survival of patients with structural heart disease and risk factors for sudden cardiac death. However, there is no consensus about how to treat patients with recurrent appropriate implantable defibrillators therapies. Recent studies have shown that radiofrequency ablation is an effective treatment for patients with recurrent episodes of ventricular tachycardia. Specifically, substrate ablation techniques have been shown to be especially useful in the case of ARVD, as these techniques allow performing ablation during sinus rhythm. Additionally, emerging tools as electroanatomic navigation, CT or MRI have provided further knowledge of the pathogenesis of ARVD, allowing the development of novel therapeutic approaches. In this review epidemiologic, pathogenic, diagnostic and therapeutic features of ARVD are discussed, with special focus on the treatment of ventricular arrhythmias associated with ARVD.