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Coronary artery anomaly and variation is a group of congenital abnormality of the coronary artery with a wide variety of clinical manifestations. The cases are rare and usually found incidentally, but they become interesting because they are the second most causes for sudden cardiac death (SCD) in young adults participating in competitive sports. The number of cases of coronary artery anomaly is starting to rise as the number of cases undergoing invasive and non-invasive diagnosis procedure. Single coronary artery is a very rare congenital anomaly where there is only one coronary ostium to supply the whole heart. This anomaly usually occurs with other congenital anomaly. The symptoms may be unspecific from asymptomatic until SCD. This case report discusses about a patient with single coronary artery, its clinical approach, and the treatment options.

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