Neurological manifestations may complicate Takayasu arteritis (TA) but seizures are rare. A 40-year-old man with TA presented with recurrent episodes of epileptic seizures. Episodes consisted of a brief period of unresponsiveness followed by sudden falling, tonic stiffening and limb jerking. A postictal period with drowsiness, urine incontinence and a temporal loss of memory was also present. A carotid and intracranial duplex ultrasound revealed a reverse flow within the left vertebral artery indicating the presence of subclavian stealing syndrome while extracranial MRA suggested some stenosis at the origin of the left common carotid artery. The EEG was consistent with epilepsy. Neurological manifestations are secondary to ischemia caused by decreased blood flow in the involved carotid and vertebral arteries. Antiepileptic treatment proved effective and may be considered as a reasonable first approach. The stenotic lesions can be managed successfully with angioplasty but these procedures are associated with a high failure rate and may not be needed.