The World Health Organization is still revising the epidemiology of multi-system inflammatory syndrome in children (MIS-C) and the preliminary case definition, although there is a dearth of robust evidence regarding the clinical presentations, severity, and outcomes. Researchers, epidemiologists, and clinicians are struggling to characterize and describe the disease phenomenon while taking care of the diseased persons at the forefronts. This report tackles the first case of a 13-year-old Saudi female with the MIS-C mimicking Kawasaki disease. Her main manifestations were fever, gastrointestinal symptoms, evidence of organ failure with an increase in inflammatory markers, and a history of coronavirus disease (COVID-19) infection. She had glucose-6-phosphate dehydrogenase (G6PD) deficiency and no significant previous history of any disease. She presented with signs of acute illness: high-grade fever (39.6°C) for five days accompanied by sore throat, malaise, reduced oral intake, abdominal pain, diarrhea, skin rash, bilateral non-suppurative conjunctivitis, and erythematous, cracked lips. Eventually, she died despite aggressive management based on the Centers for Disease Control and Prevention and the Saudi Ministry of Health guidelines for COVID-19 management. Based on this case, we suggest that pediatricians need to be aware of such atypical presentations and early referral to tertiary care is imperative for further early diagnosis and management. MIS-C is a rare yet severe and highly critical complication of COVID-19 infection in pediatrics, leading to serious and life-threatening illnesses. Knowledge about the wide spectrum of presenting signs and symptoms and disease severity, including early detection and treatment, is pivotal to prevent a tragic outcome.