Multisystem inflammatory syndrome (MIS-C) is a pediatric hyperinflammation disorder caused by severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2). It has now been reported from several countries the world over. Some of the clinical manifestations of MIS-C mimic Kawasaki disease (KD) shock syndrome. MIS-C develops 4-6 weeks following SARS-CoV-2 infection, and is presumably initiated by adaptive immune response. Though it has multisystem involvement, it is the cardiovascular manifestations that are most prominent. High titres of anti-SARS-CoV-2 antibodies are seen in these patients. As this is a new disease entity, its immunopathogenesis is not fully elucidated. Whether it has some overlap with KD is still unclear. Current treatment guidelines recommend use of intravenous immunoglobulin and high-dose corticosteroids as first-line treatment. Mortality rates of MIS-C are lower compared to adult forms of severe COVID-19 disease. Keywords Multisystem inflammatory syndrome (MIS-C) • Kawasaki disease (KD) • Hyperinflammation • Coronavirus disease 2019 • Kawasaki-like disease Background Kawasaki disease (KD) is a medium vessel vasculitis of undetermined etiology usually affecting children below 5 years [1-3]. Rowley et al. had hypothesized as far back as 2004 that an unidentified respiratory infectious agent with tropism to vascular tissue, likely a virus, could be linked to the etiology of KD [4]. This putative RNA virus, presumably a ubiquitous one, resulted in persistent infection in bronchial epithelium and macrophages and was associated with intracytoplasmic inclusions [5, 6]. Rheumatology INTERNATIONAL Jayakanthan Kabeerdoss and Rakesh Kumar Pilania are the joint first authors and they contributed equally. Disclaimer We hereby state that this manuscript is original and no part of the manuscript has either been published before, or is under consideration of publication elsewhere. The definition of scientific term alluded to, in the manuscript have been duly cited.