Long QT syndrome (LQTS) is an infrequently occurring familial disorder in which affected members have electrocardiographic QT interval prolongation and a propensity to syncope and fatal ventricular arrhythmias. This review of the current literature includes discussions of inheritance, clinical presentation, diagnosis, and treatment of LQTS. At present, there are three modalities of treatment for LQTS patients: beta-blockers, pacemakers, and left cervicothoracic sympathetic ganglionectomy. Because the clinical course of LQTS is quite variable, therapy must be individualized for each patient.