Long QT syndrome is a congenital disorder accompanied by a high incidence of sudden cardiac death. β-adrenergic blockade is the therapy of choice, and it is successful in 75–80% of patients. However, for those in whom refractory arrhythmia or cardiac events are not prevented by medication, the literature suggests that left cardiac sympathetic denervation may be useful. Here we present a girl 20 months of age with refractory ventricular tachycardia due to long QT syndrome successfully treated by left cardiac sympathetic denervation. There was no significant complication.