Introduction : Coronary artery anomalies are defined as marked deviations from the normal coronary anatomy and are detected in 1-2% of the population. Patients with coronary artery anomalies are usually asymptomatic and most anomalies are discovered incidentally during a coronary angiography. Case Report : A 63-year-old female with a history of hypertension and diabetes mellitus was referred for coronary angiography. She had been having substernal chest pains, which were aggravated by exertion and relieved by rest for several weeks. The resting electrogram demonstrated sinus rhythm without any ST segment or T wave abnormalities. A echocardiography showed a suspicious akinesia on the mid-portion of the interventricular septum and the ejection fraction was 72%. A stress echocardiography was done and was accepted to be questionably positive for ischemia on LAD territories. Cardiac catheterization was then performed via the right femoral artery using the Judkin's technique. When attempting to engage her left coronary artery using a left Judkin's catheter, another coronary artery was seen along the normal left circumflex artery, which originates from a root of the left main coronary artery or aortic wall directly. With selective left coronary angiography, there was a 90% focal stenosis at the mid-portion of the left anterior descending artery close to the bifurcation to the first diagonal branch. A selective right coronary angiography showed no stenosis or thrombus.