ARVC clusters in families and its pattern of inheritance is generally autosomal dominant with age-related, reduced penetrance.2 The discovery that Naxos disease, a rare cardiocutaneous autosomal recessive form of ARVC, was caused by pathogenic variants in JUP-encoded plakoglobin3 prompted rapid identification of pathogenic variants in other desmosomal genes (PKP2, DSP, DSG2, and DSC2) in ARVC populations. The gene discussed is DSP; the disease is Naxos disease.