We report an unusual case of a 12-month-old boy diagnosed with anomalous origin of a single coronary artery from the pulmonary artery associated with patent ductus arteriosus. The patient survival was attributed to left-to-right shunt (patent ductus arteriosus) allowing for appropriate myocardial perfusion. Successful surgical correction involved patent ductus arteriosus closure, mitral annuloplasty and reimplantation of the coronary artery into the aortic root.