Since its first description in 1992 as a new clinical entity, the Brugada syndrome has aroused great interest among physicians and basic scientists. Two consensus conferences held in 2002 and 2005 helped refine the current accepted definite diagnostic criteria for the syndrome, briefly, the characteristic ECG pattern (right bundle branch block and persistent ST segment elevation in right precordial leads) together with the susceptibility for ventricular fibrillation and sudden death. In the last years, clinical and basic research have provided very valuable knowledge on the genetic basis, the cellular mechanisms responsible for the typical ECG features and the electrical susceptibility, the clinical particularities and modulators, the diagnostic value of drug challenge, the risk stratification of sudden death (possibly the most controversial issue) and, finally, the possible therapeutic approaches for the disease. Each one of these points is discussed in this review, which intends to provide updated information supplied by recent clinical and basic studies.