Long QT syndrome is characterized by syncope and fatal ventricular arrhythmia or fibrillation at an young age. A 25-year-old female patient with congenital Long QT syndrome (Jervell and Lange-Nielsen syndrome) was scheduled for cochlea implantation due to congenital deafness. During anesthetic induction, maintenance, and emergence of these patients, cardiac arrests have been reported, which may be due to asymmetrical adrenergic stimuli in the heart, especially in unrecognized cases. Beta blocker is the first-line therapeutic drug for long QT syndrome. However, there is a controversy with regard to which anesthetics are safe for the management of patients with long QT syndrome. This case report describes an anesthetic management of a patient with congenital long QT syndrome who was treated with beta blocker.