Arrhythmogenic right ventricular dysplasia (ARVD) is a predisposing factor for sport-related cardiac arrest (CA), sudden cardiac death (SD), and life-threatening ventricular tachyarrhythmias (VT). The aim of this study was the assessment of athletes with ARVD, particularly the CA survivors. From 1974 to January 1996, 1642 competitive athletes (aver. 25.5 yr.), 136 of whom were top level athletes (TLA), were studied for important arrhythmic manifestations. All athletes underwent an individualised study protocol including a series of non invasive and invasive diagnostic techniques. One hundred and one athletes (90 males, 11 females, aver. 25.9 yr.) were diagnosed as being affected by ARVD on the basis of the WHO/ISFC criteria. The same percentage (about 6%) of ARVD is present in both the general arrhythmic athletes population and in the subgroup of TLA. Prevalence of ARVD among athletes with CA or SD is high (respectively 23% and 25%), confirming the observation that ARVD is one of the major causes of SD in Italian athletes. All CA were athletic activity related, indicating the potentiality of exercise as a cause of electrical destabilisation in subjects with ARVD. In athletes with documented ARVD intense sport activity has to be proscribed. In athletes at risk of CA or SD an aggressive treatment, ICD implantation and RF catheter ablation must be taken into consideration.