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The present case report is computed tomography (CT) coronary angiographic depiction of an exceedingly rare entity-single right coronary artery arising from the right sinus of Valsalva with the absence of equivalent left coronary artery system branches and associated mitral valve prolapse. Even though a statistical rarity, it is potentially fatal and can cause myocardial ischemia, sudden cardiac death, and warrants immediate clinical attention. Further, the report reveals the decisive role of CT coronary angiogram in the diagnosis of such rare entities, in contrast to catheter angiography, which may be inconclusive.