Introduction: We present the case of a young man with multiple atherosclerotic risk factors and a rare cause of angina and supraventricular ectopic beats. Case presentation: A 35-year-old man with one-year history of stable angina pectoris and supraventricular ectopic beats, without any medication, presented to the emergency room complaining of anterior chest pain that occurs at moderate physical exertion. The rest electrocardiogram, transthoracic echocardiography, and chest X-ray were normal; the exertion stress test was also normal, except for supraventricular ectopic beats (trigeminal rhythm). Due to the intermediate pre-test probability of coronary artery disease, he was evaluated using coronary computed tomography angiography. An anomalous origin of the right coronary artery from the left coronary sinus, with an inter-arterial course and without any atheroma plaques, was observed. During hospitalization the evolution was stable, without complications. The patient was further referred to a cardiac surgery clinic to evaluate the possibility of surgical treatment of this anomaly. Conclusions: Coronary artery anomalies are very rare; however, they present multiple implications in current practice. The most severe complication of this condition is represented by sudden death in young patients due to malignant ventricular arrhythmias. Imaging diagnostic techniques allow for a rapid, noninvasive diagnosis of this rare cause of angina.