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The total occlusion of the left main coronary artery is rarely observed (approximately 0.05% of coronary angiographic studies). We have tried to draw the clinical and pathophysiological outline of this condition, starting from our experience and reviewing the published reports. A hard selection, principally by the high mortality in patients with left main coronary stenosis, limits the number of those who present total occlusion. These subjects show a remarkable variability in their clinical presentation. It is not possible to find out a significant correlation with a single risk factor, clinical manifestation or electrocardiographic picture. Therefore, these patients cannot be distinguished from other subjects affected by severe atherosclerotic ischemic heart disease. The angiographic finding of a dominant right coronary artery is most frequent. A rich collateral circulation to the left coronary is usually observed. From a pathophysiological point of view, the efficiency of coronary collateral circulation is confirmed by the significant correlation of its extent with the left ventricular function. An important role is also played by the rate of progression of left main stenosis in total occlusion and by the presence of right coronary lesions. Even if statistical evidence is still lacking, surgical treatment is unanimously indicated and achieves satisfying results. The use of nonsurgical recanalization techniques, such as intracoronary thrombolysis and transluminal angioplasty, may be lifesaving in those patients in whom left main coronary occlusion suddenly occurs.