There is a subset of patients with neurocardiogenic syncope among whom syncopal recurrences are frequent and without prodromic symptoms, or have a traumatic outcome. For this subgroup the term "malignant neurocardiogenic syncope" has been proposed. Frequently, these patients suffer from syncopes that are accompanied by profound bradycardia and prolonged asystole (more than 5 seconds). Although in the literature the term malignant is used as equal to syncope with prolonged asystole, this practice could lead to prognostic and therapeutic errors. The present article describes its clinical features, the usefulness of head-up tilt test for its therapeutic evaluation, and the wide variety of treatments that have been proposed including beta-adrenergic blockade, DDD pacemakers, and repeated tilt training program.