We report a patient clinically diagnosed with HCM who displays LV hypertrophy but carries 2 pathogenic/likely pathogenic variants, one of β-myosin heavy chain (MYH7 p.Glu924Lys), a gene well-established as causative for HCM, and the other of plakophilin 2 (PKP2 p.Leu442Argfs∗5), the most common desmosomal gene implicated in ARVC. Here, PKP2 is linked to arrhythmogenic right ventricular cardiomyopathy.