In this study, we investigated medical history and symptoms before death in all subjects aged 1 to 35 years who died a sudden cardiac death (SCD) from arrhythmogenic right ventricular cardiomyopathy (ARVC) in Denmark in the years 2000 to 2006. All deaths (n=6,629) in subjects aged 1 to 35 years in Denmark in the period 2000 to 2006 were included. A total of 16 cases of SCD due to ARVC were identified based on histopathologic examination. Information on medical history was retrieved from The National Patient Registry, general practitioners, and hospitals. Symptoms before death were compared with 2 control groups in the same age group and time interval: one consisting of subjects who died in traffic accidents (n=74) and the other consisting of patients who died a SCD due to coronary artery disease (CAD; n=34). In the case group, 8 of the 16 patients with ARVC experienced antecedent cardiac symptoms and 7 of them sought medical attention. None were diagnosed with ARVC before death. Only 1 patient in the healthy control group and 31 of the 39 patients with CAD experienced cardiac symptoms before death. A total of 6 patients of the 16 with ARVC died during strenuous physical activity and 4 of the deaths were sports-related SCDs. In conclusion, antecedent cardiac symptoms before SCD in the young were seen in 1/2 of the patients who died because of ARVC, and this is significantly higher than in the healthy control group. When considering the ARVC and CAD groups collectively, antecedent cardiac symptoms are seen in the majority.