Epidemiological studies indicate that there is a high rate of mental retardation and behavioural problems in children with epilepsy. In some cases both the epilepsy and the mental retardation will have a common cause, such as a metabolic disorder or brain trauma. However, in other children, the epilepsy itself may cause either temporary or permanent learning problems. When permanent learning disability can be prevented it is important to treat the epilepsy early and effectively. Children with specific learning difficulties and memory problems can benefit greatly from appropriate management. There are many causes of behavioural disturbance in children with epilepsy. These causes include the epilepsy itself, treatment of the epilepsy, reactions to the epilepsy, associated brain damage/dysfunction and causes that are equally applicable to children who do not have epilepsy. Identifying the cause or causes in each child allows rational management to be provided. Antiepileptic treatment with medication or surgery can either improve the situation or make matters worse. The treatment should be tailored to the needs of the individual child. If surgery is required, there is a strong argument for performing this early in life, both to allow the greatest opportunity for brain plasticity and also to allow the child full benefit from the important developmental and educational years, without the problems that can be associated with the epilepsy. Skilled management of children with epilepsy who have mental retardation and/or behavioural problems can be very rewarding both for the family and for the professionals involved.