Atrial myxoma is a cardiac tumor often histologically benign but very insidious for its mechanical complications. Among these, myocardial infarction can be an expression of coronary embolism. Imaging techniques are essential for the diagnosis and the therapeutic steps. We describe the clinical case of a 52-year-old woman with acute myocardial infarction and normal coronary arteries with left atrial myxoma. We conducted a review of published case reports over the last 45 years on the rare association between atrial myxoma and acute myocardial infarction, to obtain pathogenic and epidemiological information from the real world.