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The early repolarization (ER) pattern has historically been regarded as a benign ECG variant. However, in recent years this view has been challenged based on multiple reports linking the ER pattern with an increased risk of sudden cardiac death. The mechanistic basis of ventricular arrhythmogenesis in ER syndrome is presently incompletely understood. Furthermore, strategies for risk stratification and therapy for ER syndrome remain suboptimal. The recent emergence of novel mapping techniques for cardiac arrhythmia has ushered a new era of research into the mechanistic basis of ER syndrome. This review provides an overview of current evidence relating to ER and risk of ventricular arrhythmias and discusses potential future areas of research to elucidate the mechanisms of ventricular arrhythmogenesis.