Moyamoya disease is a chronic cerebrovascular disease, more common in Japanese and Asian population. It is rare in European and American population. The typical presentation is seizure, Transient Ischemic Attack or Stroke and diagnosed radiologically with typical angiographic appearance of the vascular collateral network. We are reporting this unusual case of Moyamoya Disease in a Caucasian adult who presented to A&E with an episode of tonic colonic seizure. In past she had Recurrent Transient Ischemic Attacks so as a part of workup for stroke and seizure, all relevant workup was done. Carotid doppler showed an increase in end-diastolic flow velocity and a decrease in vascular resistance in both external carotid arteries. Subsequently, MRI Brain and MRA showed a rare Moyamoya disease. This disease should be considered an important differential in patients presenting with recurrent seizure episodes or neurological symptoms.