We present the case of 44-yr-old man who presented syncope with ventricular tachycardia in the setting of Brugada syndrome. In addition to the electrocardiographic evidence of the syndrome and the absence of apparent structural heart disease, clear defects of myocardial neuronal metaiodobenzylguanidine (MIBG) uptake on MIBG SPECT imaging also were found in inferior, apical and septal walls. Thallium-201 SPECT distribution was homogeneous along the left ventricle. Thus, cardiac MIBG scintigraphy provides information about left ventricular dysinnervation in a patient with Brugada syndrome, enhancing the clinical utility of myocardial MIBG SPECT imaging in life-threatening ventricular arrhythmias.