Multisystem Inflammatory Syndrome in Children (MIS-C) associated with new coronavirus infection (COVID-19), with signs of Kawasaki disease (KD) and toxic shock syndrome, well-defined diagnostic criteria, is the most severe manifestation of COVID-19 in pediatric patients. MIS-C is analogous to the cytokine storm in children with COVID-19. The article presents a clinical observation of a child with MIS-C with a lethal outcome. Clinical and anamnestic data, the results of laboratory and instrumental research allowed to diagnose MIS-C in a 2-year-old girl with full KD form. Autopsy results, detailed microscopic examination, which revealed systemic vasculitis of small and medium-sized vessels, inflammatory infiltrates in different organs, are presented, clinical and morphological comparisons are made. © 2020, Pediatria Ltd.. All rights reserved.