DSG2 and cardiomyopathy: A systematic review of all the reported ACM cases with the homozygous <i>DSG2</i> variants suggested that the additional genetic mutations contributed to the early age onset of ACM and lethal cardiac events.<h4>Conclusion</h4>In conclusion, we report three rare cases of ACM with the same homozygous <i>DSG2</i> variant in combination with additional heterozygous mutations in cardiomyopathy-associated genes.