In recent years, the early repolarization pattern has emerged as a risk factor for malignant ventricular arrhythmias and sudden cardiac death. The identification of the subset of patients who are at high risk of sudden death represents a significant challenge to the clinician. Multiple clinical and ECG features have been associated with an increased risk of sudden deathin however the majority of risk factors confer a small increase in absolute risk. The present article reviews current evidence and potential management strategies in patients with early repolarization.