We screened 808 patients diagnosed with nonischemic cardiomyopathy at Osaka University Hospital from 2011 to 2023 via exome sequence analysis and identified five patients with DSG2 (c.355 C > T, p.Arg119Ter) variants, including one patient with a previously reported homozygous variant11 and four patients with heterozygous variants. This evidence concerns the gene DSG2 and nonischemic cardiomyopathy.