BackgroundCurrently, guidelines from around the world endorse measurement of coronary artery calcium (CAC) to improve clinical risk prediction in appropriately selected asymptomatic and stable symptomatic individuals. A CAC score of zero may discourage from further testing as coronary computed tomography angiography (CCTA). We investigate the presence of malignant coronary artery anomalies (CAA)s among stable symptomatic patients with zero CAC. MethodsA total of 281 individuals' information was obtained. These individuals had low to intermediate pre-test probability of coronary artery disease, complained of stable typical or atypical chest pain, were not known to have CAD, and had CAC scan score of zero. After investigating the CCTA, Angelini's classification system for CAA was utilized in adapted form to determine the presence, the class and type of the CAA. ResultsThe CAAs were detected in 16 (5.7 %) patients on CCTA, 15 (8.1 %) of them were below 45 years. The mean age for patients with CAAs was 31.8. According to Angelini classification system, most of the detected CAAs were malignant such as the origination of the coronary artery from the opposite sinus with arterial course between the aortic and pulmonary trunks and the intramural muscular bridge course. ConclusionIt is preferable to perform CCTA in young patients with cardiac symptoms, especially in Asian and Middle Eastern countries even of the CAC score is zero.