The occurrence of neonatal seizures is an important clinical sign indicating brain disorder in neonates. An identification of neonatal seizures is critical in the management of high risk neonates. However, the diagnosis and management of neonatal seizures are challenging, because electroclinical dissociation is an outstanding feature of neonatal seizures. Neonatal seizures are frequently not accompanied by any identifiable clinical symptoms even on close observation, whereas motor phenomena which have been considered to be seizures are not associated with ictal electroencephalography (EEG) correlates. For this reason, neonatal seizures should be diagnosed based on ictal EEG findings and the efficacy of treatment should be evaluated using continuous EEG monitoring. EEG is also useful diagnosing the underlying etiology of neonatal seizures. Although conventional EEG is the gold standard for the diagnosis of neonatal seizures, amplitude-integrated EEG (aEEG) can be considered an option. However, aEEG has substantial limitations. In treatment two aspects must be considered. First, neonatal seizures themselves require emergency therapy and second, etiology-specific therapy is important in order to prevent further brain injury. At present, evidence is limited on the treatment of neonatal seizures. In order to establish effective treatment, studies using continuous EEG/aEEG monitoring and long-term follow-up are necessary. Widespread use of EEG/aEEG is desirable in order to solve several problems in the diagnosis and treatment of neonatal seizures.