Congenital absence of the left circumflex artery (CALCx) or an anomalous origin of the left circumflex artery from the right coronary artery is a unique anomaly in the literature that has been incidentally diagnosed with coronary angiography. CALCx is characterized by an angiographical absence of the left circumflex artery, with a super-dominant right coronary artery that provides the postero-lateral wall of the left ventricle. We present a review of the literature of a total of 52 CALCx cases reported so far including our case. In our study, the average age of patients was 52.83 years (median – 55 years; standard deviation – 13.05 years; range 12–76 years) with a male to female ratio of 1.93:1. The chronic coronary syndrome was the most common clinical presentation followed by the acute coronary syndrome. In 45.5% of cases, the associated coronary artery disease was documented. A comprehensive anatomical and functional assessment is required for the appropriate management strategy.