Context: Multisystem Inflammatory Syndrome in Children (MISC) is a newly and rising condition, particularly in SARS-CoV-2 high transmission communities. Objective: Analyze current literature and reported cases of MISC, concerning its clinical spectrum, complications associated, therapeutic strategies and distinguishing features of other clinical syndromes. Data Sources: Extensive literature research was performed in MEDLINE (trough PubMed), Scopus and Web of Science from December 2019 to December 2020.Study Selection: First analysis included all article titles and abstracts screening to identify relevant studies and second analysis included a full text screening of previous selected studies. Eligibility was assessed independently by two authors and disagreements were resolved by discussion and consensus. Data Extraction: Data were extracted on MISC definition, demographic data, clinical features, diagnostic tests, laboratory analysis andimaging, therapeutical approach and outcomes. Results: Common symptoms included: gastrointestinal (70%), rash (57%) and cardiovascular (52% with shock). Notable differences with Kawasaki Disease were identified including age, clinical presentation and cardiac involvement. 30% presented positive SARS-CoV-2 2 reverse transcription polymerase chain reaction and 51% positive serologies. 62% received intravenous immunoglobulin and 42% glucocorticoids. 62% required intensive care, 21 children died (<2%). Severe presentations were associated with neurological symptoms, hepatitis and acute kidney injury. Limitations: As a recently documented disease, there was limited prospective and follow-up studies, therefore disregarding long-term sequelae and prognosis. Conclusions: MISC raises concern on its severe cardiac involvement at presentation, with frequent intensive care and immunomodulatory therapy need. Short term outcomes seem to be favorable, with cardiac disfunction recovery and low mortality rates.