Coronary artery anomalies are rare but sometimes important findings in the evaluation of the coronary artery. The majority of the anomalies are of benign prognosis, but others can be associated with cardiac symptoms and syndromes (angina, dyspnea, syncope, congestive heart failure, myocardial infarction and sudden death). The potentially serious anomalies include: ectopic coronary origin from the pulmonary artery; ectopic coronary origin from the opposite aortic sinus; and large coronary fistulae. Appropriate diagnosis is critical for recognition and management. Treatment can be performed by surgical or percutaneous approach. However, management is conservative in the majority of them. Contemporary diagnosis and clinical management of these anomalies are briefly reviewed and discussed in this article.