The Bland-White-Garland Syndrome represents the anomalous origin of the left coronary artery of pulmonary trunk. Only 10% of the patients reach adulthood. Clinical manifestations of the syndrome are angina, dyspnoe, ECG signs of ischemia, myocardial infarction, and death in childhood. We present the case of a 47 year old woman with Bland-White-Garland Syndrome, who was resuscitated from ventricular fibrillation. The only symptom shown in her personal history was progressive dyspnoea in the last 6 months, though mitral insufficiency was known since childhood. On echocardiographic examination, she showed an anterolateral infarction and a mitral insufficiency II. As operation procedure, the ligation of the left main coronary artery and bypass surgery with a left internal mammarian graft to the left descending branch of the left coronary artery was chosen. The mechanism of onset of ventricular tachycardia in our patient is not known. Three pathophysiological mechanisms may be possible: (1) local ischemia caused by the shunt, (2) a reentry circuit in the border zone of myocardial infarction, (3) electrical instability caused by endocardial fibrosis. As local ischemia and reentry circuit were widely excluded, only endocardial fibrosis could induce further ventricular arrhythmia. We therefore intended to implant an AICD to have the most possible safety for our patient. But this, postoperatively was refused by the patient. In analogy to Coronary Artery Disease, the risk for sudden cardiac death postoperatively may be due to three factors: (1) presence of a reentrant circuit, (2) LV-function below 40%, and (3) presence of endocardial fibrosis. Our patient showed a low risk for sudden cardiac death. On electrophysiological study, no ventricular tachycardia could be induced in our patient, indicating the absence of a reentry circuit. LV function exceeded more than 40%. In Holter ECG, only few ventricular premature beats could be registrated, indicating a low risk for sudden cardiac death in the presence of endocardial fibrosis. In the follow-up of fourteen months, the patient remained free from arrhythmic events.