Background: Multisystemic inflammatory syndrome is a rare but severe complication in children and adolescents infected with SARS-CoV-2. Case report: This report describes a four-year-old Arab descent female presenting with a history of high-grade fever of 7 days duration with erythematous rash and family history of COVID infection suggestive of atypical Kawasaki disease, which turned out to be multisystemic inflammatory syndrome (MIS-C) post COVID infection. Based on the available published evidence, the World Health Organization has provided a preliminary definition of MIS-C as “children and adolescents 0–19 years of age with fever > 3 days presenting with two of five clinical criteria, such as 1. skin rash or mucocutaneous inflammation or non-purulent conjunctivitis, 2. hypotensive shock, 3. signs of myocardial dysfunction, 4. coagulopathy, and 5. the acute gastrointestinal problem, elevated inflammatory markers, no evidence of other microbial infections, and evidence of COVID-19 (RT-PCR, antigen test or serology positive), or likely contact with patients with COVID-19”. Case Report: Some criteria are similar, and others are different from Kawasaki disease and toxic shock syndrome, such as the presence of rash, conjunctivitis, mucocutaneous inflammation, and coronaries involvement are shared criteria between Kawasaki and MIS-C, whereas hypotensive shock and coagulopathy are shared criteria between MIS-C and toxic shock syndrome. The positive testing for COVID-19 in the absence of microbiological isolation confirms MIS-C. Conclusion: Among the complications of COVID-19 infection, MIS-C is rare. Owing to the management differences among Kawasaki, toxic shock syndrome, and MIS-C, timely diagnosis of MIS-C saves the children from a poor outcome.