<h4>Background</h4>The histopathological features of PRKAG2 cardiomyopathy have been reported in a fragmentary manner.<h4>Objective</h4>We aimed to systematically evaluate the cardiac pathological features of PRKAG2 cardiomyopathy in a large patient cohort and assess their diagnostic potential compared to genetic sequencing.<h4>Methods</h4>We conducted an observational, cross-sectional, case-control study including 18 patients with PRKAG2 cardiomyopathy and 11 heart transplant recipients as controls. Here, PRKAG2 is linked to cardiomyopathy.