ABSTRACTEnteroviruses (EVs) are a common cause of neonatal infections, and perinatal EV infection can lead to severe neonatal disease, including sepsis‐like presentations, and adverse pregnancy outcomes. However, a comprehensive understanding of the prevalence and clinical manifestations of perinatal EV infection is lacking. This systematic review investigated the prevalence and clinical manifestations of perinatal EV infection. A comprehensive literature search was performed in PubMed, Embase, and KoreaMed up to August 26, 2024. Studies describing perinatal outcomes related to EV infection in neonates and pregnant women were included. Data extraction and quality assessment were performed independently by two reviewers. Nine studies (three each from America, Europe, and Asia) were included. Severe neonatal complications included sepsis‐like disease and death. Maternal symptoms included fever, uterine contractions, and rash. Perinatal EV infection prevalence ranged from 60% to 77.8% in severely affected neonates, 25% to 57.1% in infected pregnant women, and 4.6% to 46.1% in all infected newborns. Placental infection was confirmed in 38.3% of severe neonatal cases. This review highlights the global presence and potential severity of perinatal EV infections, underscoring the need for enhanced surveillance, standardized diagnostic protocols, and further research to inform effective prevention and management strategies.