The anomalous origin of the right coronary artery from the left sinus with interarterial course is a rare but life-threatening coronary abnormality. Coronary computed tomography is crucial in identifying this disease whose treatment, based on coronary artery bypass grafting, is recommended in symptomatic patients but is more controversial in asymptomatic patients. The case report presented offers an opportunity to discuss the pathophysiological, diagnostic and therapeutic aspects of this congenital coronary artery disease.