Epilepsy is a disorder in which the balance between cerebral excitability and inhibition is tipped toward uncontrolled excitability. There is now clear evidence that there are distinct differences between the immature and mature brain in the pathophysiology and consequences of seizures. Both the enhanced excitability of the immature brain compared with the mature brain and the unique pathologic consequences of seizures are related to the sequential development and expression of essential signaling pathways. Although the immature brain is less vulnerable than the mature brain to seizure-induced cell death, seizures in the developing brain can result in irreversible alterations in neuronal connectivity. Developing novel strategies to treat and avert the consequences of seizures in children will require further understanding of the unique mechanisms of seizure initiation and propagation in the immature brain.