Several studies have described multisystem inflammatory syndrome (MIS) in children, but very few investigations presented this syndrome among neonates (MIS-N). The present study has reviewed the current knowledge about MIS-N, its etiology, symptoms, and outcomes to attract special attention with an eye on earlier diagnosis and treatment of newborns affected by perinatal SARS-CoV-2-infection. A narrative review study was conducted (Tehran, Iran, 2021). All types of full English articles (up to October 1 2021) were included. Detailed research on academic search engines was performed. The implemented Mesh-based keywords were "SARS-COVID-2" OR "COVID-19" OR "coronavirus" AND "Neonate" OR "Neonatal" OR "Newborn" AND "Multisystem Inflammatory Syndrome" OR "MIS-N" OR "inflammatory response syndrome" AND "Pregnancy" OR "Perinatal" OR "prenatal". Finally, 15 Full-text articles were included that met the eligibility criteria. Available data related to the disease, its etiology, presentations, and its outcome were collected and discussed. The cause of MIS-N is the transmission or production of SARS-CoV2 antibodies in response to SARS-CoV2 infection. By involving different organs, the clinical manifestations of MIS-N may mimic sepsis, toxic shock syndrome, RDS, Kawasaki disease, necrotizing enterocolitis, myocarditis, meningitis/encephalitis, aortic thrombosis, ETC. Besides the clinical presentations, detecting reactive anti-SARS-CoV-2 IgG antibodies could be a notable clue in MIS-N diagnosis. Supportive therapy, suppressing the autoimmune and inflammatory responses, anti-platelet agents, and anticoagulants were reported as the effective therapeutic agents to improve the outcome. The present study highlighted the possibility of MIS-N as an infrequent but severe syndrome consequent to perinatal COVID-19 infection. Although the diagnosis is still controversial, clinical suspicion, laboratory findings, and early treatment initiation could improve the outcome of this immunological disease.