SCN5A deleterious variants predispose to bradyarrhythmias and atrioventricular block, posing the challenge of pacemaker implantation in young patients.<h4>Case summary</h4>A 17-year-old girl with BrS and SCN5A p.(Glu901Lys) pathogenic variant presented with recurrent presyncope, dyspnea, and more than 3,000 second-degree atrioventricular blocks documented on 24-hour Holter monitoring. Here, SCN5A is linked to atrioventricular block.