A 23-year-old female patient was diagnosed with congenital long QT syndrome, discovered when she visited our cardiac department due to chest discomfort, because she had a prolonged QTc interval on ECG, history of syncopal attacks and seizures several times every year, and ingestion of anticonvulsants for several years. It is well known that the long QT syndrome is associated with sudden death secondary to ventricular tachyarrhythmia or fibrillation at a young age. Moreover cardiac arrests during induction, maintenance, and awakening of anesthesia of these patients have been reported, and may be due to asymmetrical adrenergic stimuli in the heart, especially in the unrecognised case. This case report describes the anesthetic management of a patient with congenital long QT syndrome, who presented for cervicothoracic sympathectomy for a more permanent control of life-threatening ventricular arrhythmias, and reviews the related literature.