A boy with group A xeroderma pigmentosum and a series of progressive neurologic complications developed versive seizures at 8 years of age. Electroencephalography at 6 years of age revealed no epileptic changes or focal abnormalities. He was seizure-free until versive seizures developed; electroencephalography revealed frequent spike discharges in the right central and temporal regions. Neurologic complications and electroencephalographic abnormalities of 34 patients with group A xeroderma pigmentosum also were assessed. Only 3 patients with xeroderma pigmentosum have been reported to have a seizure disorder. This patient is the fourth reported with group A xeroderma pigmentosum associated with a convulsive disorder. Although neurologic manifestations in group A xeroderma pigmentosum are progressive and severe, it is unknown why so few of these patients develop seizure disorders.