The clinical presentation and outcomes of coronavirus disease 2019 (COVID-19) are characterized by exceptional variability in manifestations, which depend on many factors, one of which is the patient's age. One of the severe life-threatening manifestations in adults is acute respiratory distress syndrome (ARDS), in some cases accompanied by the development of multiple organ failure (MOF). During the first two to three months of the COVID-19 pandemic, the global medical community was inclined to think that this infection is rather mild and not fatal in children. However, with the accumulation of new information, it became clear that there is a growing recognition of multisystem inflammatory syndrome (MIS-C) developing in children chronologically associated with SARS-CoV-2, and usually leading to serious consequences. The article presents the most essential epidemiological, clinical and laboratory characteristics of the syndrome, as well as discusses MIS-C pathogenesis and differential diagnosis aspects with a number of other acute conditions associated with imbalance of cytokines, and available pharmacotherapies. © 2020 Ima-Press Publishing House. All rights reserved.