Bicuspid aortic valve is a common congenital anomaly often associated with aortic valve degeneration and ascending aorta dilation, leading to stenosis or insufficiency. We present the case of a 46-year-old female with Turner syndrome, a Sievers type 1 bicuspid aortic valve, moderate aortic insufficiency and a dilated aortic root. Given her age and anatomical features, a valve-sparing aortic root replacement using the hemi-Yacoub technique was performed. This technique preserved the patient's native aortic valve, thereby avoiding the need for anticoagulation therapy. Postoperatively, no residual valve insufficiency was detected, and the patient demonstrated a normal valve coaptation height.