Conventional coronary angiography is the gold standard for the diagnosis of coronary artery anomalies. Coronary anomalies are relatively rare findings in patients undergoing conventional coronary angiography for suspected obstructive coronary artery disease. Recently, the increasing performance of diagnostic techniques, such as electron beam tomography (EBT), magnetic resonance (MR) and, more recently, multislice computed tomography (MSCT), has enabled their application to cardiac imaging. MSCT, in particular, has a prominent role in coronary imaging due to its spatial and temporal resolution and three-dimensional capabilities. We report the incidence and pathophysiology of coronary artery anomalies based on the capabilities of recent diagnostic tools with the aim of improving an accurate and noninvasive diagnostic approach.