Arrhytmogenic cardiomyopathy (AC) is an inherited heart muscle disease characterized pathologically by right ventricular (RV) fibrofatty myocardial replacement and clinically by ventricular electric instability, which may lead to cardiac arrest from ventricular fibrillation (VT), mostly in young people and athletes. AC shows a propensity for life-threatening ventricular arrhithmias during physical exercise, and participation in competitive athletics has been associated with an increased risk for sudden cardiac death (SCD). in addition, physical sport activity has been implicated as a factor promoting acceleration of disease progression. Identification of affected athletes by pre participation screening has proved to result in mortality reduction during sport activity. This article examines the role of AC in causing SCD in young competitive athletes and addresses prevention strategy based on identification of affected athletes at pre participation screening.