A 32-year-old man without risk factors for coronary artery disease but moderate cigarette smoking died suddenly 5 years after a cardiological check-up for chest pain on effort. Autopsy investigation ruled out extracardiac causes of death and pointed to a multivessel coronary artery disease with organized and recanalized occlusive thrombosis, sometimes superimposed to smooth muscle cells proliferation, in the absence of peripheral vascular disease. Thromboangiitis obliterans of the coronary arteries or accelerated atherosclerosis complicated by thrombosis due to endothelial erosion are debated as possible etiopathogenetic mechanisms. The case is still open for achieving a final diagnosis.