Abstract Introduction To the best of our knowledge, there are no previous univocal data on how CCT can accurately describe morphological variants and subtypes of congenital coronary artery abnormalities. Taking into consideration the extensive application of CCT in clinical practice in the last decades, it is not uncommon to have a congenital coronary artery anomaly detected at CCT. Thus, the study aim is to evaluate the diagnostic and prognostic impact of specific cardiac CT parameters in subjects with a diagnosis of congenital coronary artery anomalies. Material and Methods This is an intermediate evaluation of a prospective clinical registry on a population of subjects who underwent a cardiac computed tomography imaging evaluation in the period between January 2007 and October 2015 and were diagnosed to have a congenital coronary abnormality. For the present preliminary analysis only coronary anomalies of origin have been considered and 92 patients have been included. Follow-up have been collected either through a structured telephonic interview or through the evaluation of clinical records of subsequent hospitalization or ambulatory visits. Results The population enrolled in the present study has an average age of 63,0 ± 12,8 years (range 34 to 85 years), with a male prevalence of 69,6%. The left main artery is missing in 46 individuals (50%), with separate origin of LAD and LCX. Only two duplications (2.2%), one for the left coronary artery and one for the right coronary artery, have been discovered. Six participants (6.5%) had a non-coronary artery origin abnormalities and pulmonary artery was the site of origin in the majority of cases. 51 subjects (55,4%) have an anomalous origin of the coronary artery from a different coronary sinus and 50 subjects had also an anomalous course with the retro-aortic being the most common (30,4%). 11 participants (12,0%) displayed also an intramural segment and 16 (17,4%) had abnormal ostial morphology. The high take-off of the vessel was uncommon (3,3%). Age appears to be the only relevant characteristic; indeed, it displays a significant correlation both to MACE (OR 95% CI 1.03, 1.01-1.07; p=0.0349) and to all-cause of death (OR 95% CI 1.14, 1.02-1.27; p=0.0151). None of the traditional cardiovascular risk factors were found to be significantly linked with adverse outcomes in this study sample of coronary anomalies. Conclusions The result of this intermediate evaluation is that cardiac CT can be successfully used to define the anatomy and features of CAA. However, it demonstrated that in middle-aged patients, the tomographic finding of an abnormality of coronary origin might not have a meaningful, strong negative prognostic value in terms of major cardiovascular events and all-cause of death. This means that probably, in the majority of the cases, once the diagnosis is performed later on in life, no further systematic assessment is needed but a personalized approach should be suggested.