The diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia is determined according to Task Force Criteria published in 1994 that included imaging abnormalities of the right ventricle and diagnostic pathologic evaluation findings of the right ventricular myocardium by endomyocardial biopsy. These have recently been modified to include evaluation using cardiac magnetic resonance imaging. In addition, quantitative criteria for the percentage of fibrosis and the decrease in myocytes have been included in the new criteria. The pitfalls of determining the presence of arrhythmogenic right ventricular cardiomyopathy/dysplasia at autopsy and the difficulty in assessing the presence of this disease in family members are well illustrated in the present report. In conclusion, we have illustrated the need to subscribe to the modified criteria to avoid misdiagnosis.