The incidence of cardiac events in patients with variant angina pectoris without significant coronary stenosis and ST-segment elevation was analyzed during a 12-year follow-up period in 273 consecutive patients (82% men) admitted from 1986 through 2010. Among the 252 patients who underwent electrocardiography during pain, 205 had ST-segment elevation (82%) and 45 had ST-segment depression (18%). During index hospitalization, angina occurred in 179 patients (66%), ventricular tachycardia or fibrillation in 28 (10%), and complete atrioventricular block in 3 (1%), but there were no deaths or myocardial infarctions (MIs). At 140 months, angina was still present in 129 patients (47%), but frequent angina (>10 episodes/year) occurred in only 6%. Total mortality, cardiac mortality, and MI rates were 24%, 7.0%, and 6%, respectively. Cardiac death or MI occurred in 28 patients (10%), associated with tobacco smoking (p = 0.004), antecedent “first-wind” angina (p = 0.020), and angina during hospitalization (p = 0.044) and with continued smoking (p = 0.056) and recurrent angina during follow-up (p <0.001). Multivariate analysis identified age (p = 0.001), antecedent infarction (p = 0.005), first-wind angina (p = 0.009), and smoking at index hospitalization (p = 0.027) as predictors of total mortality and treatment with calcium antagonists (p = 0.047) and smoking during follow-up (p = 0.110) for cardiac mortality and MI. In conclusion, during 12-year follow-up, patients with variant angina pectoris, mostly with ST-segment elevation during pain, had a reduced incidence of cardiac mortality and MI, associated with first-wind angina, persistent angina, and continued smoking.