A case of congenital staphylococcal scalded skin syndrome (SSSS) with fatal outcome in a premature infant is reported. An intrauterine infection with Staphylococcus aureus was probably the cause for the fulminant course of the disease. Despite adequate antibiotic treatment, the infant died within 24 h after birth because of respiratory failure. Conclusion: Although rare, infection may occur in the perinatal period and SSSS may present within the first 24 h of life. In this situation, early administering of appropriate antibiotics is essential.