The paper presents the case of a 7-day-old newborn, born at term, with widespread cutaneous lesions on their limbs and trunk, sparing their face, palms and soles. The lesions, initially vesicular, evolved into crusts and hyperkeratosis, following Blaschko’s lines. Given the maternal history of similar neonatal lesions, incontinentia pigmenti (IP) was suspected, confirmed by the typical lesion distribution and absence of infections. IP is an X-linked dominant genetic disorder primarily affecting females, potentially involving other ectodermal tissues with ocular, dental and neurological abnormalities. Early recognition of cutaneous manifestations is crucial for timely multidisciplinary follow-up and definitive genetic diagnosis.