Introduction: Incontinentia pigmenti is a rare, X-linked dominant multisystem genodermatosis that presents at or soon after birth with characteristic cutaneous signs. The main features occur in the skin where a blistering rash occurs in the newborn period, followed by the blisters becoming raised-like warts. After the skin, the central nervous system is the next most affected system.
Case report: We report a female infant who was born with vesicular eruptions who was initially thought to have congenital herpes simplex virus infection.
Conclusion: This case report emphasises that incontinentia pigmenti should be included in the differential diagnosis of cutaneous blistering lesions and central nervous system involvement in neonates.