Early repolarisation was previously considered a benign variant but in recent years has emerged as a marker of risk for sudden death. In part, this appears to reflect a change in the definition. ECG territory, degree of J-point elevation and ST-segment morphology are associated with different degrees of risk for subsequent ventricular arrhythmia. At present the dataset is insufficient to allow risk stratification in asymptomatic individuals and further epidemiological and mechanistic research is required.