Sinus of Valsalva aneurysm (SVA) is an abnormal dilatation of the aortic root located between the aortic valve annulus and the sinotubular junction and is rare in the pediatric population. This case report describes a unique case of a 16-year-old adolescent patient admitted with progressive heart failure symptoms and diagnosed with a ruptured noncoronary SVA. He underwent surgical repair of the SVA with autologous pericardial patches and had an uncomplicated postoperative course. A genetic workup revealed an underlying 22q11.2 deletion that is infrequently associated with SVA.