As the ANO5-related LGE located in the right ventricular insertions and lateral wall has not been previously reported, this case underscores the importance of cardiac MRI and genetic testing in diagnosing ANO5-related dilated cardiomyopathy, revealing unique imaging patterns that expand our understanding of ANO5-associated cardiac conditions and highlights the need for advanced diagnostic and personalized treatment strategies. Here, ANO5 is linked to dilated cardiomyopathy.