Congenital coronary anomalies remain a debated issue. On the basis of a critical review of the literature and our historical series, we propose a simple clinical profile scoring system for congenital coronary anomalies. A review of literature over the past 30 years was performed, searching for a relationship between the worst coronary anomaly presentation and manifestations and the anatomical and functional features. A risk scoring system was created based on these features, and retrospectively applied to our historical series of 140 consecutive patients (52 females; mean age, 60.1±19.3 years; mean follow-up, 60±23 months). Origin from the pulmonary artery, intramural course, intramyocardial course, coronary fistula with a significant pulmonary-to-systemic flow ratio, superimposed coronary artery disease, and associated congenital heart disease were associated with the worst clinical presentation. The risk scoring system gave 2 points to anatomical features and 1 point to the association with clinical and functional characteristics: 3 risk classes were identified: >3, 2-3, and <2 points. The system showed good correlation with presentation and manifestations on follow-up. Although not exhaustive, the proposed scoring system may simplify the clinical evaluation of patients with such abnormalities, being a model for decision making.