Any coronary artery morphology which is not observed in >1% of a general population is morphologically defined as anomalous.Congenital anomalies of origin of coronary arteries are rare and usually an incidental finding.The Incidence variesfrom 0.24% to 1.3% of the cases. The most common coronary artery anomaly is left circumflex artery (LCX) arising from the right sinus of Valsalva(RSV) or right coronary artery (RCA). Other anomalies include a single coronary artery from the left sinus of Valsalva, both coronary arteries from RSV and left anterior descending coronary artery (LAD) from RSV. Majority of these anomalies are benign; however, few are potentially fatal like anomalous origin of left main from RSV or from pulmonary artery. We report a case series of three patients, who presented with acute coronary syndrome and were found to have anomalousleft main coronary artery (LMCA) arising from the RSV on diagnostic angiogram. We alsodescribe the review of literature of similar cases with an emphasis on managementand prognosis of this rare anomaly.