We present a case of a rarely seen anomalous circumflex (C x) artery originating from the proximal right coronary artery. This was an incidental finding during a diagnostic coronary angiogram performed to investigate a patient with acute coronary syndrome. Surgically significant disease was present in four left-sided vessels. The patient underwent coronary artery bypass grafts using left internal thoracic artery and long saphenous vein grafts to all four left sided vessels including the aberrant C x. We also present a discussion of current relevant literature and guidelines in relation to definition, classification, incidence, diagnosis, clinical relevance and treatment of this type of coronary artery anomalies.