Coronary anomalies are found in less than 1% of diagnostic coronary angiograms. The clinical relevance of these anomalies varies from insignificant to potentially lethal. Here, we present a rare case of a 46-year-old male who was previously diagnosed with severe aortic regurgitation with sub-aortic ventricular septal defect with coronary cameral fistula. Coronary angiography and 2-D echocardiography were showing that this fistula was connecting from the left circumflex artery to the left atrium (LA). Intra-operatively, there was no coronary cameral fistula; it was just an anomalous coronary artery arising from the left circumflex artery and ending upon the left atrial wall. This rare entity should be discussed; as in the literature, anomalous coronary in a case of ventricular septal defect with aortic regurgitation has not been reported yet.