Introduction: Incontinentia Pigmenti (IP) is a rare X-linked dominant disorder affecting ectodermal tissues, often misdiagnosed in the neonatal period. We present a rare case, highlighting the multitude of symptoms and diagnostic challenges seen in early infancy . Case presentation: A day 3 baby girl presented with a generalized erythematous rash with occasional blisters, initially thought to be extensive erythema toxicum. The following day she developed short lasting convulsions that were brought under control with anti-seizure medications on day 5. The mother had no risk factors for sepsis, no previous miscarriages, and the antenatal and post-natal periods were uneventful. There was no family history of metabolic diseases. Septic screen was normal, but markedly elevated eosinophils were seen, with giant cells visualized on skin scrapings. PCR for HSV 1 & 2, VZV , and JEV IgM were negative. Ultrasound scan brain revealed cerebral oedema but no structural abnormalities. Over the next few days the rash evolved into a typical vesicular rash, followed by a hyperpigmented rash along lines of Blaschko. The typical skin manifestation, together with a high eosinophil count favoured a clinical diagnosis of Incontinentia Pigmenti. At 18 months of age, she is thriving well but has had 3 episodes of severe sepsis. There is no dental eruption, and she persists to have hypopigmented streaks. Conclusion: Incontinentia Pigmenti is an extremely rare X-linked dominant genetic disorderdue to mutations in the IKBKG gene, causing ectodermal manifestations evolving through 4 stages. Severe cases may involve the nervous and immune system as in this patient.