Multisystem inflammatory syndrome in children (MIS-C) is a severe inflammatory syndrome diagnosed as Kawasaki-like disease manifested by toxic shock and cardiogenic or vascular paralytic shock. MIS-C usually has atypical onset age (older) and ethnic background (non-Asian). MIS-C is a severe condition and can progress rapidly and deteriorate in a short time. MIS-C is potentially associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV- 2 ). Since the pathogenesis of MIS-C is unclear, the treatments of MIS-C are based on treatment strategies in Kawasaki disease or coronavirus disease 2019 (COVID- 19 ), and it is reported some of new treatment strategies are effective. This article reviews the status and progress of diagnosis and treatment for MIS-C.