Sudden cardiac death in the young is a relatively uncommon but marked event usually related to congenital diseases or anomalies. Despite the prevalence of each condition being variable, most common causes include primary myocardial diseases and arrhythmic disorder, frequently with inheritance pattern. Sudden cardiac death is usually preceded by symptoms, thus making personal and family history fundamental for its prevention. Nevertheless, in more than 50% of cases, sudden cardiac death is the first manifestation of the disease. In this review, we describe the different causes of sudden cardiac death, their incidence, and currently used preventive strategies.