The tissue distribution of amiodarone and desethylamiodarone, its active metabolite, was examined in an autopsied case, an 85-year-old man, with arrhythmogenic right ventricular cardiomyopathy (ARVC) who had been receiving amiodarone for 4 years. High concentrations of amiodarone, a very highly lipophilic compound, were found in adipose and bone marrow tissues. Despite a low dose (100 mg daily), the concentrations of amiodarone were higher in the epicardial fat (570.4 microg/g) and in the right atrium (165.3 microg/g) than previously reported in patients with other cardiac diseases. The ratios of amiodarone/ desethylamiodarone concentrations in both tissues were >1. This unique distribution demonstrates the involvement of histological characteristics of ARVC, such as fatty replacement of myocardium. We anticipate that a low dose of amiodarone would be effective in preventing arrhythmia in some patients with ARVC.