Background: Epidermal necrosis in a neonate is an uncommon event with a variety of potential cases.
Result: We report a case of intrauterine epidermal necrosis in a preterm infant, with death occurring soon after birth. The histopathology of the denuded skin revealed full-thickness epidermal necrosis and calcification within both the epidermis and follicular structures.
Conclusion: We believe this represents the fourth reported case of lethal intrauterine epidermal necrosis and follicular calcification.