The bicuspid aortic valve (BAV) is the most frequent congenital valvular disease affecting up to 2% of the general population. BAV patients are at high risk of developing progressive aortic valve dysfunction, mainly aortic stenosis in elderly patients and aortic incompetence in younger subjects. In such patients also ascending aorta or root dilatation is often associated caused by hemodynamic and genetic factors with a progression which is not yet well defined. Since BAV patients are at risk of aortic events, such as aortic dissection or rupture, they warrant frequent and close monitoring while elective surgical intervention is the only effective method to prevent severe aortic-related complications. Repair techniques for BAV and associated aneurysm have evolved markedly with initially excellent early and medium-term results with less evidence of stable repairs in the long-term. However, over the years, predictors and mechanisms of failure were identified allowing to be specifically addressed at the time of operation, thus contributing to improve repair durability. This chapter summarizes the current principles and late outcomes or surgical repair in patients with either isolated BAV or BAV with associated aortopathy.