Being born with a bicuspid aortic valve (BAV) is a significant risk factor for developing an ascending aortic aneurysm (AscAA). Research has uncovered different mechanisms influencing AscAA development in BAV-patients compared to those with normal tricuspid aortic valves (TAV). BAV-associated AscAA may result from intrinsic hemodynamic or genetic alterations, possibly even embryonic origins. During embryonic development, neural crest cells and the second heart field contribute to the ascending aorta’s formation, with defective signaling potentially increasing susceptibility to aneurysm development. BAV can manifest with different phenotypes, impacting clinical outcomes. The degenerative AscAA in TAV-patients differs from BAV-associated AscAA, marked by fibrosis, smooth muscle cell loss, and inflammation. AscAA in TAV-patients rarely appears in those with aortic stenosis, suggesting a link between aortic valve disease and degenerative AscAA. This chapter aims to describe suggested molecular mechanisms driving aneurysm formation in BAV- and TAV-patients.