Abstract: Objective We aimed to analyze the onset age and genotype of CHD2-related epileptic encephalopathy and to improve the understanding that the disease can occur in the neonatal period. Methods We analyzed one newborn with epileptic encephalopathy admitted to Children's Hospital of Fudan University. We searched the gene "CHD2" in HGMD database and key words including "CHD2 gene" in CNKI, VIP database, Wanfang database, PubMed and the Web of Science database. All of the databases were searched up to Nov 29, 2018. Results The male newborn in the hospital mainly presented with seizures, dystonia and feeding difficulties. A de novo mutation［NM_001271:exon31:c.3951G>C(p.L1317F)］was detected in CHD2 gene. We reviewed 67 cases from Chinese and foreign literature databases. None of the reported cases had seizures during the neonatal period. Sixty-one point one percent (11/18) of cases showed developmental delay before the onset of seizures. According to the reported cases and the case from this study, we found that all of the three cases who presented with seizures within 6 months (including 6 months old) carried missense variants of CHD2 (3/3，100%), and 5 of 19 cases (5/19，26.3%) occuring seizures at the age of older than 6 months carried missense variants. Conclusion The defected CHD2 can cause epileptic encephalopathy in the neonatal period. The missense variants of CHD2 may have a tendency to cause early epilepsy. Defected CHD2 should be taken into consideration for patients with neurodevelopmental disorders and no seizures.