In this report we describe a patient with the left circumflex coronary artery originating from the distal right coronary artery as a terminal extension of it. Other origin anomalies related to the circumflex coronary artery may be associated with sudden death and adverse cardiac events, but no data are available about the clinical consequences of this rare anomaly. We also report the clinical follow-up of this patient over a 13-year period, with anomalous circumflex coronary artery arising as a terminal extension of the right coronary artery.