Takayasu arteritis is a granulomatous vasculitis of medium- to large-sized arteries. Initial presentation is usually with nonspecific constitutional symptoms, but unusually, it can present with acute ischemic features in the advanced disease. We present a case of a young Asian female who presented with acute stroke due to Takayasu arteritis. Involvement of proximal arteries was a source of embolism, which was the most likely cause of stroke in this patient. Patient made a good clinical recovery with a combination of medical treatment and rehabilitation. Takayasu arteritis should be considered as a possibility in all young patients presenting with stroke, as this is fairly responsive to medical management and associated with good clinical outcomes.