Congenital absence of left circumflex coronary artery (LCX) is a very rare congenital anomaly of the coronary circulation, and few cases have been reported in the literature. We report a case of a 48-year-old male who had congenital absence of LCX presented with vasospastic angina. Routine coronary angiography showed congenital absence of the LCX, typical myocardial bridge in the mid left anterior descending coronary artery (LAD) and super-dominant right coronary artery (RCA). The dominant LAD showed a spastic appearance at the baseline coronary angiography and significant spasm was induced by incremental intracoronary acetylcholine (ACh) provocation test at mid LAD where the myocardial bridge site was observed and was extended to the distal LAD. Typical anginal chest pain was documented during the Ach provocation test and the patient was stabilized with conventional anti-anginal medication. Precise morphological and functional evaluation of the anomalous coronary artery is important for selecting the best treatment modality and better prognosis.