Neuroacanthocytosis is a group of disorders, clinically characterized with movement disorders, self-mutilation and seizures. There is little information in the literature regarding the clinical and EEG findings of the accompanying seizures in this neurodegenerative disorder. A 46-year-old man who was diagnosed as chorea-acanthocytosis, a subgroup of neuroacanthocytosis, was investigated for severe drug resistant seizures. Continuous video-EEG monitoring revealed an active left temporal epileptogenic focus with left temporal rhythmic discharges detected during his complex partial seizures. T2-weighted cranial MRI indicated hyperintensity of the left amygdala as well as hyperintensity and atrophy of bilateral basal ganglia. We discuss the development of focal seizures in a patient who has a neurodegenerative disease, namely neuroacanthocytosis.