The early repolarization pattern as long been regarded as a benign ECG variant. However in recent years, compelling evidence has emerged from a number of studies to suggest that early repolarization is associated with an increased risk of 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 death in 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.