Abstract. Kernicterus caused by severe jaundice is a devastating condition and has long-term neurodevelopmental consequences in children. Infections and drugs can trigger severe hemolysis in newborns with deficiency of glucose-6-phosphate dehydrogenase (G-6-PD), and can precipitously increase the severity of jaundice. In this case report, we speculate that Staphylococcus aureus sepsis may have triggered severe hemolysis in a G-6-PD-deficient preterm neonate, leading to severe neonatal jaundice and kernicterus. To our knowledge this is the first such reported case.