SUmmARY A 14-year-old boy had suffered from intermittent acute hepatic porphyria, myoclonic convulsions, and mental retardation (LennoxGastaut syndrome). The porphyria was treated by stopping the administration of phenobarbitone and phenytoin. Sodium valproate at a dose of 70 mg/kg per day lessened the severity and frequency of convulsive crises.Acute intermittent porphyria (AIP) is rare in children, but is not unknown. In a survey of reports on such children, Barclay (1974)