Moyamoya is a progressive vaso occlusive disease of large intracranial arteries with characteristic collaterals formation. It is more frequent in females compared to males.lt has a bimodal age of onset at around age 5 and at around age 40.The most common initial presentation is Transient ischaemic attack (TIA) is also a frequent initial presentation and maybe recurrent. The symptoms tend to clear rapidly but recurred in some instances. Less commonly,they may present with headache, convulsions, impaired mental clarity, visual disturbance, and nystagmus. In older patients, subarachnoid hemorrhage is the most common initial manifestation. We report a case of a 38 year old female patient diagnosed with Moyamoya disease