Left main coronary artery (LMCA) thrombosis with acute myocardial infarction is an uncommon condition with an extremely high mortality. The small number of reported cases prevents the development of an evidence-based approach. Hence there are no clear-cut guidelines describing the best management approach for this condition. We describe our experience with six patients who presented with LMCA thrombosis and discuss the epidemiology, etiology and management options available for this high-risk subgroup.