Abstract:Epilepsy is the most common neurological disease in childhood, and genetic abnormality is one of the most common causes. The surgical treatment in children with genetic epilepsy has made rapid progress in recent years. This paper summarizes and reviews the pediatric genetic epilepsy and its surgical indications, the evaluation of the relationship between genetic abnormality and epilepsy and selection of surgical approaches, resection surgery for pediatric genetic epilepsy, neuromodulation surgery for pediatric genetic epilepsy, and other surgical methods. It is concluded that genetic abnormality will not become a contraindication in the surgical treatment of pediatric genetic epilepsy, but will only become an important consideration in the preoperative evaluation. Resection surgery and vagus nerve stimulation are the most common used surgical approaches for genetic epilepsy in childhood. Surgical treatments can reach favorable outcomes in rational selected children with intractable genetic epilepsy.