Abstract: Epileptic encephalopathies are age-dependent conditions, typically drug resistant, characterized by severe cognitive impairment due to the epileptic activity itself. Corticosteroid therapy with adrenocorticotrophic hormone, hydrocortisone, prednisone or methylprednisolone is one of the therapeutic approaches tried in these children. Although the first use of steroids as antiepileptic drugs dates back to 1950, there is no wide agreement about the ideal treatment regimen in epileptic encephalopathies other than West syndrome; no evidence from class I and II studies are available at the moment. In this study, we present a literature review on steroid therapy in childhood epilepsy with special regard to effectiveness, tolerability and possible mechanisms of action.