Multisystem Inflammatory Syndrome in Children (MIS-C) is a hyperinflammatory disorder resulting from infection with severe acute coronavirus-2 respiratory syndrome (SARS-CoV-2). It usually affects the pediatric population with the highest prevalence in children between the ages of three and 12, and the most common comorbidities are obesity and asthma. MIS-C develops four to six weeks after SARS-CoV-2 infection, and in most cases the presence of SARS-CoV-2 cannot be detected by PCR. The syndrome is characterized by fever, inflammation and multiorgan dysfunction and numerous similarities with other inflammatory and autoimmune conditions. The most prominent manifestations of MIS-C are symptoms of the cardiovascular system, and include myocarditis and heart failure. Due to the high incidence of cardiovascular complications, echocardiography is of great value in diagnosing and monitoring the course of the disease and assessing the severity of cardiovascular involvement. MIS-C is characterized by a hyperinflammatory condition and the onset of a cytokine storm, which can be demonstrated by laboratory markers of inflammation. Elevated values of the specific cardiac marker troponin indicate direct injury to myocardial cells, and significant increases in BNP and related peptides indicate exacerbation of interstitial myocardial edema and resulting vasculopathy. Laboratory findings of anemia, leukopenia, lymphopenia, thrombocytopenia and hypoalbuminemia are also common. Severe cases of MIS-C lead to the development of vasodilatory or cardiogenic shock requiring urgent hospitalization in the intensive care unit. Intravenous immunoglobulin (IVIG), glucocorticoids and biological agents are the main therapeutic modalities for the treatment of MIS-C. The use of IVIG and high doses of corticosteroids is indicated as first-line therapy for the treatment of MIS-C. The second line of therapy includes antiplatelet drugs and biologics such as anakinra, tocilizumab or infiximab, especially in the case of refractory diseases on the application of the first line of therapy.