Introduction: Brugada syndrome (BrS) with potassium channel mutation is less frequently reported. We report the case of a 26-year-old male with unexplained syncope who was discovered to have a novel KCNH2 gene mutation with right ventricular structural abnormalities. Methods and Results: A type I Brugada EKG pattern was revealed with V1 and V2 leads moving to the second intercostal space. Echocardiography and cardiac MRI demonstrated right ventricular structural abnormalities and myocardial fibrosis. Genetic testing identified a novel heterozygous mutation in the KCNH2 gene. The treatment method involves the implantation of an implantable cardioverter-defibrillator (ICD). After 23 months of ICD implantation, his arrhythmia was terminated by ICD with evidence of ventricular fibrillation. Νo new arrhythmia recorded during follow-up. Conclusion: A deeper understanding of BrS is required to establish a potential role for KCNH2 gene mutation and right ventricular structural abnormalities in generating ventricular arrhythmias.