Background: Bicuspid aortic valve (BAV) is the most frequent congenital heart disease affecting 1% to 2% of the population, with thoracic aortic aneurysm (TAA) formation being its second most frequent complication after aortic valve dysfunction (stenosis or insufficiency). Aortic valve replacement (AVR) with mechanical or biologic prostheses has long been the procedure of choice. Although prosthetic AVR is effective in correcting the hemodynamic problem, there are important long-term drawbacks. These are particularly present in a younger patient population, such as the majority of individuals with a BAV. Patient and methods: 40 patients underwent aortic valve repair for bicuspid aortic valve disease with ascending aortic aneurysm in the period between June 2020 and February 2022 in Kasr Alainy hospitals. Results: All our patients showed significant improvement regarding left ventricular dimensions and contractility. Two patients had mild to moderate regurge and thirty eight patients had non-significant regurge in the six month follow up echo. One patient complicated by complete heart block required permanent pacemaker implantation, another two patients had postoperative high drainage required revision for bleeding. One patient died from ventilator associated pneumonia after prolonged mechanical ventilation due to disturbed conscious level.