Congenital absence of the left circumflex artery (LCX) is a very rare congenital anomaly of the coronary circulation, and only a few cases have been reported in the literature. We report on a 55-year-old female with atypical chest pain. Routine coronary angiography showed a normal left anterior descending coronary artery (LAD), no LCX and a dominant right coronary artery (RCA), which continued beyond the crux, running the full course of the LCX and terminating in the left atrial branch. Neither aortography nor pulmonary angiography showed a separate ostium for the LCX. There were no atherosclerotic lesions in the coronary arteries, or ischaemia on stress myocardial perfusion imaging. Multi-detector row computed tomography (MDCT) was performed to confirm the diagnosis.