Incontinentia pigmenti is a rare disorder that may affect many systems including the skin, central nervous system, bone, and eyes. We describe a 13-day-old girl who developed seizures on day 1 of life and was placed on antiseizure medication. On approximately day 4 of life, she developed a vesicular rash on her trunk and extremities. The pediatric team prescribed intravenous acyclovir and diphenhydramine cream. These were used without improvement. At dermatology consultation, linear and swirled vesicular lesions were seen. A skin biopsy specimen revealed eosinophils within intraepidermal vesicles consistent with a diagnoses of incontinentia pigmenti. This case of incontinentia pigmenti is of interest in that the initial symptom was a seizure disorder.