The presence of an anomalous origin and/or distribution of a coronary artery as the infarct-related vessel during primary angioplasty for acute myocardial infarction might represent a clinical and technical challenge. We report on a case of a patient with an acute anterior myocardial infarction who was referred to our hospital for primary angioplasty and whose culprit lesion was located on an aberrant left main coronary artery. The purpose of this case report was to review the incidence and angiographic evaluation of an anomalous coronary vessel and to discuss the technical approach to an anomalous left main intervention and its impact on clinical outcome.