The coronavirus disease 2019 (COVID-19) has afflicted individuals of all ages, which has a high rate of morbidity and fatality. Although most children with COVID-19 infection experience minor symptoms, a growing proportion of COVID-19 infections in children are linked to a novel multisystem inflammatory syndrome with Kawasaki disease-like clinical features. Multisystem inflammatory syndrome in children (MIS-C) is a novel hyperinflammatory syndrome that can impact about any organ system. The most frequent symptoms are fever and gastrointestinal disorders, although neurologic and dermatologic manifestations are also well-documented. The clinical symptoms of MIS-C coincide with those of Kawasaki disease, toxic shock syndrome, and shock syndrome, making diagnosis challenging. Elevated inflammatory markers are common in MIS-C patients, and an abnormal echocardiogram or electrocardiogram may be present. Intravenous immunoglobulin, anticoagulation, and corticosteroids should all be considered in the treatment of MIS-C patients. Even those with significant cardiovascular involvement, the majority of patients recover without complications. Echocardiographic follow-up is required as coronary aneurysms have been documented. This narrative review is to review the epidemiology, pathophysiology, clinical presentations, laboratory findings, diagnostic criteria, and treatment options for MIS-C patients in order to increase pediatricians’ knowledge of this novel syndrome.