An 8-year-old Chinese girl had a first episode of symptomatic partial epilepsy at age 2 years. Despite regular treatment with antiepileptic drugs (AEDs), the seizures remitted and exacerbated. Epileptiform EEG discharges were evident, arising from the left centroparietal region. Magnetic resonance imaging (MRI) and computed tomography (CT) scan of the temporal bone and cerebral angiography demonstrated absence of the left internal carotid artery and ipsilateral bony carotid canal. Perfusion brain single photon emission CT (SPECT) with [99mTc] HMPAO showed a perfusion defect at the left posterior temporoparietal lobe. Brain ischemia due to agenesis of the left internal carotid artery (ICA) was speculated as the cause of the partial epilepsy.