The ketogenic diet (KD) is a special diet with high-fat, low-carbohydrate and moderate amount of protein. The KD therapy is a safe, potentially effective and important treatment of refractory childhood epilepsy in recent years. It has confirmed clinical efficacy and a generally good prognosis. The adverse reactions of KD treatment are normally less and transient. In this paper, the effective rate, patient compliance, adverse reactions and their causes of KD in treating children with refractory epilepsy were reviewed.