Anomalous origin of right coronary artery is a rare congenital anomaly that was 1st described in 1948 by White and Edwards. It is well established that an anomalous origin of right coronary artery may be asymptomatic but it can lead to angina pectoris, myocardial infarction and sudden death, even in the absence of atherosclerosis. So, although many of these anomalies are clinically insignificant but some are associated with serious morbidity. Incidence of coronary anomalies in patients undergoing coronary angiography varies from 0.64% to 1.3%. We described the case of corresponding author, who had history of breathlessness off and on in the early morning in lying down posture relieved on seating posture; and once a moderate pain along the medial aspect of his right arm and then the evaluation revealed an anomalous right coronary artery arising at a higher level. We discussed the clinical importance of this anomaly and reviewed the literature concerning current views and therapy. Conceptual and therapeutic debates remain. Aim of present study is to reappraise the concept of anomalous origin of coronary artery and to discuss the potential hazards and treatment rationale of this anomaly on the basis of literature review. A comprehensive literature review was made.