Anomalous aortic origin of the coronary arteries (AAOCA) is a rare congenital abnormality. It is usually asymptomatic and often found incidentally during coronary angiography. However, it can also be discovered during the autopsy of young healthy adults who have suffered from sudden cardiac death (SCD). AAOCA represents the second most common cause of SCD in young athletes. Herein, we report a case of a 39-year-old patient with left-sided right coronary anomaly with multiple high-risk features who presented with life-threatening symptoms for SCD but normal electrocardiography, echocardiography, and cardiac markers. The coronary computed tomography revealed an anomalous coronary artery from the left sinus of Valsalva with a hypoplasic origin and a high-risk path between the aorta and the pulmonary artery with a short intramural path. He was surgically managed with a coronary artery bypass with an uneventful follow-up.