A case of a 51-year-old woman with symptoms of non-ST-segment elevation acute coronary syndrome and concomitant atrial flutter is presented. Patient underwent atrioventricular septal defect repair in childhood. Coronary angiography showed total occlusion of left main coronary artery and massive collateral network originating from right coronary artery supplying entire left coronary artery. Ablation of atrial flutter had been performed and patient was subsequently submitted to mitral valve replacement, tricuspid valvuloplasty and coronary artery bypass grafting. The potential causes of left main occlusion are in this case discussed.