Bicuspid aortic valve (BAV) is a common congenital cardiac anomaly, characterized by the presence of two functional aortic valve leaflets instead of the usual three. It affects approximately 1 – 2% of the population and is often associated with other cardiovascular complications, including aortic stenosis, aortic regurgitation, and aortic root dilation. BAV can present with a wide range of clinical manifestations, from asymptomatic individuals to those with severe aortic valve dysfunction or aortic dissection. The diagnosis of BAV is commonly established through non-invasive imaging techniques such as transthoracic and transesophageal echocardiography, with advanced modalities, such as computed tomography and magnetic resonance imaging providing superior delineation of valve morphology, aortic root measurements, and associated pathology. Management of BAV is multifaceted and depends on the severity of valvular and aortic involvement. Lifelong surveillance is often necessary, with serial imaging to monitor disease progression. Surgical intervention is indicated for patients with severe valve dysfunction, symptomatic disease, or aortic aneurysm exceeding established threshold diameters. Common procedures include aortic valve replacement (AVR) and composite graft repair, such as the Bentall or valve-sparing David procedure. Transcatheter AVR has also emerged as a less invasive alternative to surgical valve replacement within this patient population, with its indications expanding significantly in recent years due to growing interest and advancements in the field. Despite substantial progress in diagnostic and therapeutic strategies, BAV poses clinical challenges due to its heterogeneous presentation, genetic underpinnings, and the requirement for tailored management approaches.