Coronary artery anomalies (CAAs) represent one of the most confusing topic in cardiology and affect approximately 1% of the general population. Although some anomalies seem to be only anatomical curiosities, others may sometimes have fatal consequences. This review describes the anatomical characteristics of main CAAs and focuses on the pathophysiological mechanisms by which CAAs may cause a pathological state. The last section describes these therapeutical options of this congenital disorders.