Bicuspid aortic valve (BAV) disease is becoming increasingly respected by clinicians. This enhanced appreciation is based on recognition of how common and virulent this disease really is. This disease is now known to be the most common congenital lesion affecting the human heart (with potential competition in frequency posed by mitral valve prolapse). It is estimated that the BAV lesion alone accounts for more morbidity and mortality than all the other congenital cardiac lesions combined. Many authorities feel that this disease is so virulent that every individual with a BAV will, given enough time, develop aortic stenosis, aortic insufficiency or aortic aneurysm/dissection related to the bicuspid valve disease. This review looks in detail at clinical issues related to BAV disease, including genetics, pathophysiology, diagnosis, management and surgical decision making. The picture emerging from basic and clinical studies is of a defect in collagen metabolism affecting not only the aortic valve, but also the wall of the aorta itself. Timely intervention for bicuspid-related aortic valve disease or aneurysm can preserve both duration of life and quality of life in affected individuals.