Epilepsy is one of the most common neurological conditions affecting the pediatric age group, with an estimated incidence of approximately 45 per 100,000 children per year 1,2 . Long-term, population-based follow-up studies in pediatric epilepsy have shown favorable outcomes in the majority of cases, with between two-thirds and three-quarters achieving seizurefreedom, and approximately half of children having remission of their epilepsy, meaning they are able to discontinue antiepileptic medication and remain seizure-free 3,4 . However, in approximately 20% children with new-onset epilepsy, seizures will become medically intractable.Epilepsy is not a single disorder, but rather describes a diverse group of seizure types, and an even more diverse group of etiologies. There is a minority of specific etiologies or electroclinical syndromes that have uniquely efficacious therapies. This paper will summarize the clinical manifestations, diagnostic work-up and therapy of some of these specific causes that are crucial to identify. Additionally, newer anti-epileptic medications (AEDs) and dietary options for pediatric epilepsy will be reviewed.Accurate classification of epilepsy is imperative to understand its natural history, pathophysiology and to decide on best therapy. A revised classification of epilepsy has recently been published 5 , and classifies seizure disorders based on mode of onset, etiology and electroclinical syndrome or constellation. Mode of onset is divided into generalized (bihemispheric), focal (unihemispheric) or unknown, and is important to identify as several medications exacerbate specific seizure types. Some of the sodium channel agents (carbamazepine, oxcarbazepine, phenytoin) and GABA-ergic medications (vigabatrin or ABSTRACT: Epilepsy affects approximately 45 per 100,000 children per year. While many cases respond favorably to antiepileptic therapy, approximately 20% will prove to be medically intractable. This paper reviews some of the recently identified important metabolic and autoimmune etiologies for which there are specific therapies. Additionally, newer antiepileptic medications, including rufinamide, lacosamide, retigabine, eslicarbazepine and brivaracetam and newer dietary options, including the modified Atkins diet and low-glycemic index diet are discussed.