Congenital abnormalities of the coronary arteries are found in 0.6-1.5% of patients in coronary angiographies and most of them are benign. Their presence may prolong the time of the examination which is important especially in patients with acute coronary syndromes (ACS). The incidence of coronary artery anomaly with critical stenosis in the anomalous vessel may impose technical problems during percutaneous coronary intervention (PCI). We report the case of a patient with one of the most common coronary anomaly and ACS treated with multivessel PCI.