Single coronary artery (SCA) is a rare congenital anomaly in which the entire coronary system arises from a solitary ostium. Anomalies of the coronary arteries are rare conditions that are often asymptomatic. However, if perfusion by the coronary artery is impaired, this anomaly can lead to a life-threatening situation, such as myocardial ischemia or infarction, and sudden cardiac death. We report a case of single coronary artery in a six year old girl who presented with acute chest pain.DOI: http://dx.doi.org/10.3126/njr.v3i2.9616 Nepalese Journal of Radiology Vol.3(2)July-Dec, 2013: 81-84