The Brugada syndrome is an autosomal dominant genetic disorder with variable expression characterized by abnormal findings on the surface electrocardiogram. It is associated with an increased risk of ventricular arrhythmias and sudden cardiac death. The ECG findings consist of right bundle branch block and persistent ST segment elevation in leads V1 and V2. Most clinical manifestations are related to life-threatening ventricular arrhythmias, most often ventricular fibrillation or polymorphic ventricular tachycardia. Patients may also present with palpitations, atrial fibrillation, syncope, or sudden cardiac arrest. Many factors may induce the manifestation of typical ECG finding, including fever, sodium channel blockers, drugs, and certain clinical conditions. The diagnosis is often made following a clinically significant event in which the patient has the typical ECG finding. Treatment is primarily focused on prevention of sudden cardiac arrest and termination of any ventricular arrhythmias by implantation of a cardioverter-defibrillator (ICD). Drugs as quinidine and isoproterenol may also be used to treat Brugada syndrome, as well as radiofrequency catheter ablation.