Multisystem inflammatory syndrome in children, also known as MIS-C, is a rare but serious illness that can appear around a month after a child recovers from COVID-19 or has been exposed to SARS-CoV-2. MIS-C causes inflammation in multiple organs and organ systems and is manifested by persistent high fever with possible organ failure due to shock caused by vasculitis. Other signs and symptoms of this syndrome can be nausea, vomiting, diarrhea, abdominal pain, lethargy, neck pain and rash. Considering the wide range of expressed symptoms and their intensities, the differential diagnosis of multisystem inflammatory syndrome in children is extensive, so it is necessary to exclude severe acute COVID-19, bacterial sepsis, toxic shock syndrome, appendicitis, Kawasaki disease, systemic lupus erythematosus, macrophage activation syndrome, infections with other viruses or rickettsiae. The cause of the syndrome is still unknown, but it is known that clusters of new cases appeared 2 to 6 weeks after the peak incidence of SARS-CoV-2 infection in the population. In its pathophysiology, MIS-C is similar to Kawasaki disease and macrophage activation syndrome, and all three conditions are hyperinflammatory, meaning there is a dysregulation of the immune response. Therapy of multisystem inflammatory syndrome in children consists of low doses of methylprednisolone and intravenous immunoglobulins if the child isn’t showing any signs of myocardial involvement. If so, the child is not given IVIG because those put an additional volume load on the heart. Instead, high pulse doses of methylprednisolone are applied with subcutaneously applied anakinra, an antagonist of the receptor for interleukin 1, as step-up therapy. Despite the similarity of the mechanism of inborn errors of immunity that cause autoimmune and autoinflammatory disease and MIS-C, their association has not been confirmed so far.