A 45-year-old male presented to the Emergency Department with a 2-h history of sudden onset right-sided headache and difficulty walking. Initial non-contrast computed tomography (CT) of the brain was unremarkable. Twelve-lead electrocardiogram showed left ventricular (LV) hypertrophy with strain pattern. On echocardiography, the aortic valve was noted to be bicuspid, associated with at least moderate eccentric aortic regurgitation and moderate aortic stenosis. In view of the initial presentation of ataxia and headaches, and the finding of bicuspid aortic valve (BAV) on echocardiography, an urgent CT of aorta and carotid arteries was performed, revealing dissection of the right vertebral artery and a right lateral medullary infarct. Aortic root dilatation and aortic dissection are well known complications of BAV. However, vertebral artery dissection in the context of BAV with a normal aortic root is unusual, and to our knowledge has only been reported once in the literature so far.