Anomalous origin of the left coronary artery connected to the pulmonary artery (ALCAPA) is a rare congenital defect with a high mortality rate in infancy if left untreated. It may cause myocardial ischaemia and can lead to myocardial infarction, mitral dysfunction, cardiac arrhythmias, heart failure and sudden death. Without operation, survival into adulthood is rare. We report clinical findings, diagnostic characteristics and therapy in a 31-year-old woman with ALCAPA and preserved left ventricular function.