The review is dedicated to matters related to epidemiology and pathogenesis of multisystem inflammatory syndrome associated with SARS-CoV-2 in children (MIS-C). The majority of the reviewed reports are focused on immunopathogenesis of the disease. The causes of the syndrome related to the features of the virus are listed in the paper, the association with circulating variants is described. The role of the SARS-CoV-2 surface protein as superantigen is considered. The literature data on the likelihood of MIS-C development according to the antibody-dependent enhancement pattern are discussed. The factors of cellular and humoral immune response contributing to hyperinflammation are addressed. Sporadic papers describing genetic mutations that can play a certain role in the MIS-C pathogenesis are provided. Furthermore, the association of vaccination against novel coronavirus infection with the likelihood of MIS-C in vaccinated individuals is discussed.