PURPOSE: The pandemic caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) has infected over 43 million people resulting in over 1 million deaths. Approximately 2% of cases in the United States are children, and in most cases the child is either asymptomatic or has mild symptoms. However, some pediatric cases can present with Multisystem Inflammatory Syndrome (MIS-C). Understanding the epidemiology, clinical presentation, and management of MIS-C related to SARS-CoV-2 will help to streamline early diagnosis and treatment, particularly in pediatric patients with complex medical conditions. METHODS: This scoping review adopted methods from the Joanna Briggs Institute (JBI) manual for evidence synthesis and preferred reporting items for systematic reviews and meta-analyses extension for scoping reviews (PRISMA-ScR) guidelines. Primary studies of patients meeting the Centers for Disease Control and Prevention (CDC) criteria for MIS-C from December 31st, 2019 to Oct 5th, 2020 were identified using PubMed and Scopus. Articles were screened for eligibility, and data collection was conducted on those fulfilling inclusion criteria. RESULTS: Of 417 studies identified, 57 met inclusion criteria, accounting for 875 patients from 15 countries. Globally, 57% of children affected with MIS-C were males. The median age was 9 years old, ranging from 6 months to 21 years. Forty-five percent of the patients had underlying comorbidities including obesity and lung disease. Fever, conjunctivitis and GI symptoms were common. Most MIS-C patients had high biomarkers including troponin I, N-terminal prohormone of B-type natriuretic peptide (NT-proBNP), D-dimer, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), white blood cells (WBCs), interleukin 6 (IL-6), procalcitonin, and ferritin. The treatment for most patients included IVIG and inotropic support. CONCLUSION: MIS-C can be a unique and potentially life-threatening manifestation of SARS-CoV-2 in children and often requires medical intervention.