Prolongation of the QT interval is a known risk factor for syncope, seizures and sudden cardiac death. Most patients with QT prolongation have an acquired cause, but congenital forms of QT prolongation are being increasingly recognized. However, existing advanced cardiac life support (ACLS) treatment algorithms for prolonged QT mediated ventricular fibrillation pertains to acquired long-QT syndrome (LQTS). Here, a young patient with out-of-hospital cardiac arrest secondary to congenital LQTS illustrates critical exceptions to the current ACLS treatment algorithms for ventricular fibrillation and unstable ventricular tachycardia when QT prolongation is congenital in origin. A clarified ACLS algorithm is proposed.