Brugada syndrome is an ion channel disease which is associated with an increased risk of sudden cardiac death. Most probably the pathogenesis of ventricular fibrillation in these patients is a combination of both genetically determined repolarisation abnormalities and conduction delay in the right ventricular epicardium. The highest risk of sudden cardiac death is present in patients who have experienced syncope before, who reveal the pathognomic electrocardiographic changes already at rest and who have inducible ventricular fibrillation. Asymptomatic patients who have the J point elevations only after administration of a sodium channel blocker seem to be at lower risk. Most recently the latest joint consensus recommendations of the largest societies for diagnostic criteria, indications for genetic testing and therapy have been published.