BACKGROUND: Abnormal separation of right coronary artery (RCA) from the left coronary system is an extremely rare variation among coronary artery anomalies. The compressions on the anomalous route of this artery may lead to arrhythmia, chest pain, or left ventricular dysfunction or may enhance formation of atherosclerotic plaques. CASE REPORT: Here, we have reported a patient presented with heart failure who had an anomalous atherosclerotic RCA originating from left anterior descending artery. Interestingly, neither the anomalous origin nor the atherosclerosis was the cause of the patient’s problems and she suffered from a hypertensive cardiomyopathy. CONCLUSION: This reminds that encountering an anomaly should not solely be interpreted as the cause of cardiac disease.