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Mutations in <jats:italic>PRKAG2<\/jats:italic> cause a cardiac syndrome comprising ventricular hypertrophy, pre\u2010excitation, and progressive conduction\u2010system disease, which is typically not diagnosed until adolescence or young adulthood. However, significant variability exists in the presentation and outcomes of patients with <jats:italic>PRKAG2<\/jats:italic> mutations, with presentation in infancy being underrecognized. The diagnosis of PRKAG2 can be challenging in infants, and we describe our experience with three patients who were initially suspected to have Pompe disease yet ultimately diagnosed with mutations in <jats:italic>PRKAG2<\/jats:italic>. A disease\u2010causing <jats:italic>PRKAG2<\/jats:italic> mutation was identified in each case, with a novel missense mutation described in one patient. 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