<p>Single coronary artery (SCA) anomalies, in which the entire coronary circulation arises from a solitary ostium, are exceedingly rare congenital variants with variable clinical implications depending on their morphologic course. Recognition of malignant trajectories, particularly interarterial courses, is critical given their association with myocardial ischemia and sudden cardiac death (SCD). This study aimed to describe the anatomical diversity, clinical presentations, and prognostic implications of SCA originating from the right sinus of Valsalva (RSV), with emphasis on the diagnostic value of coronary computed tomography angiography (CCTA). Eight patients with SCA arising from the RSV were retrospectively reviewed. Clinical presentations, associated cardiac findings, and imaging characteristics were evaluated. All patients underwent CCTA for comprehensive anatomical delineation, with invasive coronary angiographic correlation available in selected cases. Anomalies were categorized according to the modified Lipton classification. CCTA demonstrated four principal SCA courses: prepulmonic, retroaortic, interarterial, and transseptal. One patient had a bicuspid aortic valve, and several exhibited concomitant atherosclerotic disease. Some variants were associated with exertional symptoms or acute coronary syndromes, whereas others were incidentally detected and considered more benign. Management was individualized based on anatomical subtype and clinical presentation. SCA anomalies originating from the RSV exhibit substantial structural and clinical heterogeneity. Identification of high-risk features is essential for accurate risk stratification. CCTA offers superior three-dimensional visualization, enabling precise classification and guiding tailored clinical management of these rare coronary anomalies.</p>