Early repolarization (ER) pattern has been recognized for several decades and was interpreted as a variant of the normal electrocardiogram (ECG) as it was frequently observed in young healthy subjects or athletes. It is characterized by a J point elevation and ST-segment elevation inscribed as a QRS slurring or a notch of the S wave in the inferior leads or/and the lateral leads. The ER pattern has been the subject of increased interest since the report of its higher prevalence in subjects resuscitated from cardiac arrest related to idiopathic ventricular fibrillation (VF). Furthermore, population-based studies showed in healthy young adults that ER pattern was associated with an increased cardiovascular mortality and total mortality. A relationship between ER pattern and malignant arrhythmias is also supported by the experimental work of Antzelevitch et al. which provided the cellular and ionic basis for the J point elevation and its arrhythmogenic potential. The ER pattern may coexist with a number of cardiac or extracardiac conditions such as hypothermia. But this review will focus attention on the “isolated ER pattern” in healthy individuals. Antzelevitch and Yan proposed because of a number of similarities between the “ER syndrome” and the Brugada syndrome to group both syndromes under the heading of “J wave syndromes”. The management of ER syndrome (associated with idiopathic VF) is clearly the insertion of an implantable cardioverter defibrillator (ICD). The ER pattern associated with symptoms such as syncope or a familial history of sudden cardiac death requires a complete work-up. Caution should be raised not to generate anxiety in the subject with asymptomatic “isolated ER pattern” as the odds of developing malignant ventricular arrhythmias or to suffer sudden death in this case are extremely low.