Arrhythmogenic right ventricular dysplasia (ARVD) is a progressive, genetically determined fibro-fatty infiltrative myocardial disease with an estimated prevalence in the general population to be 1:5,000 to 1:10,000. ARVD leads to electrical instability that may predispose to life-threatening ventricular arrhythmia, heart failure, and sudden death. We reviewed the pathological substrate for ventricular arrhythmias, ECG findings and treatment modalities in ARVD. Importantly, novel techniques such as electroanatomic and voltage mapping has greatly improved the identification of the scared substrate in the settings of ARVD and have improved safety and efficacy of VT ablation procedures associated with this entity.