Arrhythmogenic right ventricular cardiomyopathy is characterised by progressive, fibrofatty replacement of myocardium, and ventricular arrhythmias, and its prognosis is usually poor. Arrhythmogenic right ventricular cardiomyopathy associated with atrial septal defect is very rare, and this combination may make the diagnosis, treatment, and prognosis difficult. We present a case of a patient with this association who underwent interventional treatment with a septal defect occluder. Transcatheter closure of atrial septal defect in a patient with arrhythmogenic right ventricular cardiomyopathy is hitherto unreported. During a 3-year follow-up he remained relatively stable. We also review the cases reported in the medical literature describing this uncommon association between arrhythmogenic right ventricular cardiomyopathy and atrial septal defect or patent foramen ovale.