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Refractory epilepsy, estimated to affect 10–20 % children with epilepsy, can have profound effect on the education, social and cognitive functioning and recreational activities of the child. The definitions are still evolving. A detailed clinical evaluation may reveal an accurate syndromic and etiological diagnosis. The recent advances in neuroimaging and electrophysiology have revolutionized the management of children with refractory epilepsy and supplement the clinical evaluation. Genetic and metabolic evaluation may be indicated in selected cases. The rational use of anti-epileptic drugs, epilepsy surgery and dietary therapies are the mainstay in the management. Various experimental treatment options and pharmacogenetics offer hope for future.