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.