Brugada syndrome is associated with a considerable risk of sudden death in young and otherwise healthy adults. The syndrome is estimated to be responsible for at least 4% of all sudden deaths and at least 20% of sudden deaths in patients with structurally normal hearts. The diagnosis of Brugada syndrome is based on peculiar electrocardiogram (ECG) abnormalities classified by the European Society of Cardiology in three types: type 1 (coved-type) is the diagnostic pattern; type 2 (saddle-back type); and type 3 are considered significant if there is a conversion to a type 1, spontaneously or during administration of class I A/C anti-arrhythmic drugs (flecainide, etc.). There is a general agreement about the high risk of sudden death in patients with previous cardiac arrest, for whom an implantable defibrillator (ICD) is recommended. In contrast, controversy exists on the correct clinical behaviour in individuals without a history of previous cardiac arrest. To stratify the risk in patients with type 1 pattern, three major factors have been suggested: typical ECG pattern in the basal state; a history of syncope; and inducible ventricular tachycardia/ventricular fibrillation during electrophysiological study (EPS). However, the indication and usefulness of an EPS is debatable. In patients with a type 2 or 3 pattern a pharmacological test is indicated in the presence of symptoms or of a familial history. With regard to sports eligibility, patients with a history of cardiac arrest should have an ICD and they can practise (low intensity) sport only after the implant of the device. Patients without documented cardiac arrest but at high risk (basal type 1 ECG pattern, syncope and/or positive EPS) should also have an ICD and they can practise (low intensity) sport only after the implant of the device. In patients at low risk (type 1 ECG pattern in the absence of symptoms, without family history and negative EPS) the behaviour regarding sport eligibility is not a matter of debate. In cases with type 2 or 3 pattern, in the absence of familial history and symptoms, a permissive behaviour should be assumed.