Takayasu arteritis (TA) is an uncommon disease of young women, characterized by granulomatous vasculitis of medium and large arteries. In addition to constitutional symptoms, it causes various clinical morbidities, such as arm claudication, decreased arterial pulses, carotidynia and hypertension. The incidence rate of TA has wide variation in different geographical areas. It was calculated to be 0.12 cases/100,000/ year in Sweden, 0.22 in Kuwait, 0.26 in USA, and probably higher in Japan. Neurological involvement is reported in only a minority of patients and occurrence of neurological syndromes as the first manifestation of disease has been rarely reported. We present clinical, laboratory and imaging findings of a 50 years old lady with TA, who was initially presented by clinical manifestations mimicking a stroke. Of particular importance is the occurrence of intracranial arterial stenosis in the patient, which is a relatively rare condition. The rarity of the disease and especially such a presentation can cause considerable delay in the diagnosis and treatment.