Congenital absence of the right coronary artery (RCA) is a rare coronary anomaly. Few cases of this condition have been reported. Congenital absence of the RCA is considered as a benign anomaly. However, in certain cases, these patients may develop life-threatening clinical complications that include acute myocardial infarction, stroke, or sudden death. We report two patients who were diagnosed with congenital absence of the RCA and presented with acute myocardial infarction. We discuss our experience in diagnosis and treatment of this disease. Congenital absence of the RCA with acute myocardial infarction is an uncommon clinical emergency. Therefore, early detection, correct diagnosis, and appropriate treatment are important.