Although pathogenic mutations underlying ARVC were detected in RyR2 (cardiac ryanodine receptor) [3] and in TGFβ3 (transforming growth factor-β3) [4] genes, finding mutations in genes encoding desmosomal proteins, namely Desmoplakin (DSP), Plakophilin-2 (PKP2) and Desmoglein-2 (DSG2) [5-7] led to current idea that ARVC is due to desmosomal dysfunction [8]. Here, DSG2 is linked to arrhythmogenic right ventricular cardiomyopathy.