Clinical manifestation of Brugada syndrome (BrS) mainly results from polymorphic ventricular arrhythmias and includes sudden cardiac arrest (SCA). The Brugada sign, besides being present in true BrS, may result from different causes. Moreover, electrocardiogram findings in some clinical situations may resemble the BrS electrocardiographic pattern. Thus, differential diagnosis is crucial in the proper management of patients suspected of having BrS. Lifestyle modifications and close follow-up with or without pharmacologic treatment and/or implant able cardioverter-defibrillator placement constitute the most common approach to managing BrS patients. However, the role of ablation in BrS treatment is increasing. Due to diagnostic and therapeutic difficulties, the management of BrS is often challenging. This review provides new concepts and algorithms in the diagnostics and treatment of patients suspected of having BrS.