Objective: The purpose of this pictorial essay is to review the multi detector computed tomography (MDCT) coronary angiography appearance of congenital coronary anomalies [CCA]. CCA might also be classified as hemodynamically significant or insignificant. The clinical symptoms may include chest pain, dyspnoea, palpitations, syncope, cardiomyopathy, arrhythmia, infarction and sudden cardiac death. Although CCA are relatively uncommon, they’re the second most typical reason for sudden cardiac death among young athletes and so warrant detailed review. Familiarity with atypical anatomy and their clinical presentation may facilitate appropriate diagnosis and management. This will be of immense help to the clinician planning interventional procedures like stenting, balloon dilatation, or graft surgery particularly when there are secondary changes of calcification, plaque formation and stenosis.

Conclusion: Increasing use of MDCT for cardiac imaging has helped within the detection of the many benign congenital coronary anomalies (CCA), but a little number is related to myocardial ischemia and sudden death. Increasing the employment of MDCT in cardiac imaging may yield diagnostic information on CCA not obtained with invasive coronary angiography. Axial sections, multiplanar reconstructions, virtual angioscopy, and 3D volume-rendered images should aid within the detection and improve the interpretation of such anomalies, which might be of immense help to the clinician planning interventional procedures.