A 39-year old female suddenly fell into a state of unconsciousness. She had no significant past medical history. A computed tomography scan of the head demonstrated a massive left putaminal haemorrhage with a ventricular perforation, low density areas in the right frontal lobe, corona radiata and occipital lobe. A single emergency burr hole drainage of the haematoma was performed. Bilateral common carotid arteriograms showed stenosis of the right internal carotid artery and a complete obstruction of left internal carotid artery which were both accompanied by moyamoya vessels. The biochemical studies indicated serological positive findings for RF and MPO-ANCA. She was transferred to another hospital for nursing care in a vegetative state on the 163rd hospital day. This case indicates that immunological factors, inflammation or vasculitis might have possibly been associated with the development of either an obstruction or stenosis of the intracranial internal carotid arteries.