Tehran University of Medical Sciences Immunology and Genetics Journal 2645-4831 9 1 2025 12 22 222 222 Shirin Mohamadi Division of Neonatology, Department of Pediatrics, Shohadaye Tajrish Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Fahimeh Abdollahimajd Department of Dermatology, Shohada-e Tajrish Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Soheila Sotoudeh Department of Dermatology, Children's Medical Center, Center of Excellence, Tehran University of Medical Sciences, Tehran, Iran 2025 12 15 2025 12 15 Incontinentia Pigmenti (IP) is a rare X-linked dominant genetic disorder primarily affecting the skin, with cutaneous manifestations present in all cases. Other ectodermal tissues—including the central nervous system, eyes, hair, nails, and teeth—may also be involved to varying degrees. In this report, we present the case of a newborn female who exhibited widespread vesiculopustular skin lesions at birth, predominantly affecting the upper and lower extremities. At 9 hours old, the newborn with skin lesions suspicious for generalized impetigo, transferred to the NICU at Children’s Medical Center in Tehran, Iran, for further evaluation and management. No abnormalities were observed in the hair, nails, oral mucosa, eyes, or central nervous system during the initial assessment and the final diagnosis was IP. https://igj.tums.ac.ir/index.php/igj/article/view/222