Two probands (family 1 and 3) underwent clinical work up in the outpatient cardiology clinic after DSG2 and MYBPC3 variants were reported: no cardiomyopathy was detected by echocardiography, EKG and holter-monitoring were also normal, and there was no relevant family history or reported symptoms, why regular follow-up is planned in 3–5 years. The gene discussed is DSG2; the disease is cardiomyopathy.