Background: Coronary Artery Aneurysms (CAA) are defined as localized coronary artery dilations more than 1.5 times the diameter of the adjacent segments. Giant CAA (GCAAs) are rare coronary diseases and the presence of multiple CAAs, located in more than one coronary artery, is even more uncommon. The pathophysiology is unknown but the majority are due to atherosclerosis. There is currently no consensus concerning the management. Case presentation: We report the case of a 72-year-old man, referred to our emergency department due to chest pain. The patient’s electrocardiogram was consistent with myocardial infarction with inferior leads ST-segment elevation. Two‐dimensional echocardiography showed hypertrophic Left Ventricle (LV) with akinesia of the infero-postero‐lateral LV wall and hypokinesia of the remaining LV segments (LVEF = 40%). Coronary angiography and computed tomography revealed multiple CAAs, some of them giant, associated with significant coronary artery disease: two CAAs on the right coronary artery, including a giant one (Dmax 11 mm and Dmax 32 mm); one large aneurism on the circumflex artery (D max 20.5 mm) and one on the left main descendent artery (D max 8 mm). The patient had a previous history of hypertension, dyslipidemia and abdominal aortic aneurysm. He had no history of percutaneous coronary intervention or cardiac surgical procedures. Serological studies for vasculitis or connective tissue disease were all negative. The patient underwent surgical exclusion of the giant right CAA with ligation of the proximal and distal ends of the aneurysm and double bypass surgery to the obtuse marginal and right posterior descending coronary arteries.Conclusions: CAAs’ treatment options include medical treatment, interventional or surgical management. No clear evidence about the better option exists, but it seems that surgical option should be favored in cases of bigger, more tortuous and more calcified CAAs. Based on these evidences and the anatomic lesions of our patient, we opted for surgery and he successfully underwent CAAs resection with coronary bypasses. We reported our experience and we examined the current knowledge on CAA/GCAA. Overall, management is hampered by lack of evidence. We need prospective studies and registries to further our knowledge in the management of this disorder and prognosis. From our attitude, surgical treatment should be performed for multiple GCAA to prevent fatal complications.