The risk of sudden death in patients with Brugada syndrome (BS) is still unclear. Moreover, particular clinical conditions may have a confounding effect on the diagnostic and therapeutic approach. We report the case of a 27-year-old man with a clinical history of suspected neurally mediated syncope and typical ECG features of BS. The tilt table test showed a type I, mixed, positive response. The electrophysiological study (EPS) disclosed a peculiar ventricular irritability with the induction of a life-threatening arrhythmia. After the implantation of a cardioverter-defibrillator an episode of ventricular fibrillation during sleep at night was correctly identified and treated by the device. The association between neurally mediated susceptibility and the typical ECG abnormalities of BS is not an unexpected event in young subjects. The misjudgment of the pathophysiological mechanism of syncopal episodes may lead, on one hand, to overlook the risk of sudden death and, on the other, to pursue inappropriate therapeutic measures. The application of a tailored diagnostic work-up based on currently available guidelines may be useful to overcome the clinical and therapeutic dilemma.