The symptoms of Takayasu arteritis (TA) are related to end organ ischemia. Here we present a patient with convulsions and intracranial involvement. A 15-year-old young woman was admitted with the complaint of convulsions since one and a half months previously. Her physical examination showed absent pulses and unobtainable blood pressure in both arms. Electroencephalography was normal. An arcus aorto-abdominal aortography, performed for TA prediagnosis, revealed that the subclavian artery ended as a stump at its origin on the right and was occluded by tapering on the left. Arcus aorta was normal. The right renal artery was occluded up to 80-90%. Magnetic resonance imaging of the brain revealed abnormal signal intensity in the deep white matter bilaterally. Cerebral catheter angiography showed focal stenosis of cerebral vessels; it was classified as type V according to the classification of the Takayasu Conference of 1994. A diagnosis of TA was made and 1 mg/kg steroid was given, and after a month methotrexate (15 mg/week) was added. On the tenth treatment day her pulse could be revealed. During the control period she had no convulsion. In young patients TA should be kept in mind as a rare cause in convulsion etiology. Intracranial involvement of TA must be evaluated especially if there is a headache and convulsion.