A s part of the workup for pulmonary hypertension, a 59-year-old man underwent coronary angiography. This demonstrated a single coronary artery arising from the right sinus of valsalva, which gave off 3 branches that supplied the entire left ventricle. Left ventricular function was normal by ventriculography. Angiography is often sufficient to define coronary anomalies; however, we found coronary computed tomography angiography useful to confirm this anomaly.Single coronary artery anomaly is rare, with incidence estimated at approximately 0.03%, and approximately 20 possible variations of single coronary artery have been described. Of these, antemortem reports of single coronary artery arising from the right sinus of valsalva are exceedingly rare. This anomaly would be classified according to Lipton's scheme as a variant of RII-A. A well-known case of this condition was that of a professional basketball player, who died suddenly while playing pick-up basketball. In that case, autopsy showed a single coronary artery arising from the right sinus of valsalva and a dilated cardiomyopathy. Prognosis of individuals with single coronary artery is unclear and no guidelines for treatment of this condition exist. Revascularization is recommended only if there is significant atherosclerosis and documented ischemia.