Valve-sparing aortic root replacement is one of the effective repairs for aortic regurgitation resulting from progressive dilatation of the aortic root late after surgical correction of conotruncal anomaly. However, feasibility and effectiveness of this procedure are unknown for unrepaired physiology. A 32-year-old man presented with unrepaired tetralogy of Fallot (TOF) and moderate aortic regurgitation with severe left ventricular dysfunction. Valve-sparing aortic root replacement and TOF repair were simultaneously performed. Six months after the surgical procedure, aortic regurgitation remained trivial. Concomitant valve-sparing aortic root replacement with TOF repair can be feasible and effective for unrepaired TOF with aortic regurgitation.