Background Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection has been spreading rapidly around the world,while “multisystem inflammatory syndrome in children” (MIS-C) is a new type of syndrome that has now been reported in many countries.Similar and different characteristics between KD and MIS-C have been reported in a variety of literature.We aimed to focus on reviewing clinical presentations,diagnosis,and treatment of KD and MIS-C.Methods We searched articles in the electronic databases,including the Cochrane Library database,EMBASE,and MED-LINE with the keywords “multiple inflammatory syndrome” and/or “COVID-19” and/or “Kawasaki disease” and “children”.Results Main presentations of MIS-C and KD include fever,rashes,mucous membrane involvement,conjunctivitis,hands and feet erythema/edema,and cervical lymphadenopathy.However,compared with the highest incidence of KD among some Asian countries,MIS-C is common among Black and Hispanic children.MIS-C is common in older children and teenagers,whereas classic KD is common in children under five years of age.Gastrointestinal symptoms,shock,and coagulopathy are common in MIS-C patients but are not common in classic KD.Cardiac manifestations are more common than KD,includ-ing myocarditis with cardiac dysfunction and coronary artery dilation or aneurysms.Severe cases in MIS-C present with vasodilated or cardiogenic shock that requires fluid resuscitation,muscular support,and even mechanical ventilation and extracorporeal membrane oxygenation (ECMO),whereas KD rarely presents with these manifestations and requires these treatments.Increased serum ferritin,leukopenia,lymphopenia and thrombocytopenia are common in MIS-C.However,thrombocytosis is a characteristic feature of KD.Intravenous immunoglobulin (IVIG) and moderate-high dose aspirin are still a standard recommended treatment for KD.In addition to the above-mentioned medications,steroids and biological drugs are frequently used in patients with MIS-C.Most of the children with KD have a good prognosis;however,the long-term clinical outcomes of MIS-C are not clear.Conclusions The overall presentation and treatment of MIS-C appear to overlap with KD.However,there are still great differences between the syndromes,and it is controversial to say whether MIS-C is a new entity or is a “severe type” of KD.