Sudden cardiac death (SCD) is defined as an unexpected natural death from a cardiac cause within a short time period, generally less than or equal to 1 hour from the onset of symptoms, in a person without any prior condition that seems to result in instantaneous fatality. Although such a rapid death process is attributed to cardiac arrhythmia, arrhythmia often represents the final common event in a series of events precipitated by known (95%) or unknown (5%) cardiac disorder. Electrocardiographic early repolarization involving the inferolateral leads, which was labeled benign until recently, is the latest of the primary electrical cardiac diseases discovered to have significantly high prevalence in SCD cases. Careful evaluation of patients having early repolarization associated with unexplained syncope, family history of SCD, or idiopathic ventricular arrhythmias is recommended.