A 3-year-old boy with familial long QT syndrome type 2 presented with recurrent syncope despite adequate beta-blocker therapy. Two family members had experienced sudden cardiac arrest, and one other relative had experienced sudden cardiac death. Given the high risk for ventricular arrhythmia/syncope, the decision was made to perform primary cardiac denervation therapy through a minimally invasive approach without concomitant automatic cardioverter-defibrillator implantation. Using video-assisted thoracoscopic surgery, the left-sided sympathetic ganglia from T2-T5 were identified, and dissection along the sympathetic chain with transection of the corresponding rami along T2-T5 in addition to the lower half of the stellate ganglion was performed. The chest tube was removed on day 1 after surgery, and the patient was discharged on postoperative day 4. During 14 months of follow-up evaluation, no intervening episodes of ventricular arrhythmia or syncope and no symptoms of Horner's syndrome were noted.