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Coronary artery aneurysms are a relatively infrequent finding with an incidence of 1% to 2% per year. Its cause can be atherosclerosis, congenital or due to other causes less common. Its initial manifestation can be myocardial infarction and sudden death as a result of rupture or distal embolization. The large coronary aneurysms, non-atherosclerotic, located in the common part of the left main coronary artery are exceptional. The diagnostic method of choice is the coronary angiography; however, non-invasive techniques such as transthoracic including tridimensional mode and transesophageal echocardiography, magnetic resonance imaging and computed tomography may have an important role in the detection and follow-up of these anomalies. The natural history of coronary aneurysm is not quite known. We present the case of a patient of 44 years, following an acute coronary event was diagnosed with an aneurysm in the left main and antiphospholipid syndrome. The patient received conservative treatment on the basis of antiplatelet and anticoagulant without presenting major cardiovascular events or other complications in 12 years of follow-up.