The first onset of cardiac event of long QT syndrome (LQTS) was at young age and caused by emotional or physical triggers. We presented a 64-year-old woman who experienced recurrent ventricular arrhythmia after hemodialysis initiation because of end-stage renal disease. Persistent prolonged QTc interval and diagnosis of inherited LQT2 were missed at her first 3 years of hemodialysis. The patient was beta-blocker nonresponder for ventricular arrhythmias suppression and experienced multiple ICD discharge. We reported an inherited LQT2 case with uncommon clinical manifestations and the successful experience of mexiletine use in such a patient.