Introduction: A novel missense mutation in the DPP6 gene (c.2240T > C/p.L747P) has recently been identified and linked to early repolarization syndrome (ERS). This study aims to develop a cellular model to probe variant pathogenicity. Methods: This study recruited a typical ERS patient carrying missense variant (c.2240T > C/p.L747P) in DPP6 gene (ERS) and 1 healthy control subject (CON). We generated ERS and CON induced pluripotent stem cells (iPSCs) from peripheral blood mononuclear cells through cell reprogramming. Subsequently, we used directed differentiation to create purified populations of ERS and CON iPSC-derived cardiomyocytes (iPSC-CMs). Results: Whole-cell patch clamp recordings revealed that ERS iPSC-CMs increased the transient outward potassium current (Ito) (103.97 ± 18.73 vs. 44.36 ± 16.54 mV, p = 0.04), and increased Ito density compared with CON iPSC-CMs. Furthermore, ERS iPSC-CMs demonstrated increased triggered activity, greater action potential amplitude (APA) (103.35 ± 0.61 vs. 93.84 ± 2.31 mV, p = 0.003), longer action potential duration (APD) (137.35 ± 4.59 vs. 212.20 ± 7.39 ms; 173.51 ± 5.43 ms vs. 268.96 ± 7.80 ms, 210.49 ± 5.98 vs. 341.99 ± 9.59 ms, respectively, p < 0.001). However, there were no significant difference in resting membrane potential (REP), current-voltage relationship of Ito, inactivation of Ito traces, and inactivation recovery time of Ito traces between the two groups. Conclusion: Our research highlights the cellular mechanisms underlying ERS. We observed significant electrophysiological phenotypes, including a higher incidence of triggered activity, greater APA, longer APD, and increased amplitude and density of Ito in ERS iPSC-CMs