Neurocardiogenic convulsive syncope as a disease at the border between cardiovascular and neurologic dysfunction can pose considerable diagnostic challenges. We report on the case of a 19 year-old female patient with recurrent neurocardiogenic convulsive syncope where the time from the onset of symptoms to the correct diagnosis and initiation of an effective therapy spanned more than three years. Based on this case report, we discuss differential diagnosis, pathophysiology and therapy of this disorder of autonomic cardiovascular regulation. Neurocardiogenic convulsive syncope should be considered whenever a patient has both syncope that exhibits a typical cardiovascular pattern (e.g., fainting of short duration with rapid reorientation phase) and prolonged loss of consciousness with characteristic neurological features (e.g., cerebral seizures with postictal state of confusion). Head-up tilt testing, introduced into clinical practice in 1986, is an efficient tool to diagnose neurocardiogenic syncope with comparatively high sensitivity in patients with recurrent syncope of unknown origin. Besides orthostatic training and pharmacotherapy, permanent dual-chamber cardiac pacing has gained increasing importance as treatment for cardioinhibitory forms of neurocardiogenic syncope.