Major advances have been made in the diagnosis, evaluation and management of children with epilepsy over the past 15 years. There has been a marked increase in genetic diagnoses of a number of key childhood-onset epilepsy syndromes, such as Dravet syndrome, which has been linked to mutations in the SCN1A gene. The reorganization and reclassification of epilepsies, devised by the International League Against Epilepsy, has stimulated specialists to reassess their diagnostic practices; however, many studies have not addressed the global issues in treating children with epilepsy-specifically, the challenges of diagnosis through to optimal, and appropriate, therapeutic management. Also, Class I evidence-based data that are needed as a foundation for the development of treatment guidelines worldwide are lacking. Epilepsy is common, and the impact of this disease crosses age ranges and should be managed at all levels of care from community to quaternary care. In this Review, existing data and new therapeutic management approaches are discussed with the aim of highlighting the incidence of standard practices that may not be based on clinical evidence.