Congenital long QT syndrome (LQTS) is a group of ion channel disorders of ventricular myocytes due to genetic mutations. The main symptoms of LQTS are pre-syncopal or syncopal attacks and cardiac arrest. Anti-adrenergic therapy with beta-blockers has been the mainstay of treatment. Left cardiac sympathectomy has also been used as an alternative in those in whom beta-blockers failed. ICDs are highly effective in preventing mortality and should be the first choice of therapy for those with cardiac arrest as the first symptom or those with LQT3 genotype not responding to anti-adrenergic therapy alone. Research on new therapeutic options, specifically those targeting at defective genes ormutant ion channels, may provide more effective treatment for this rare but potentially fatal electrophysiological disorder.