Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a kind of heart muscle disease characterized by the gradual replacement of the right ventricular myocardium with fibrous tissue and fat. It could be the major cause of sudden cardiac death with ventricular tachycardia, and there is a variation in the history of the disease. We reported an unusual case of ARVC in a middle-aged woman with congestive heart failure as her first presentation for a long time.