Introduction: Incontinentia Pigmenti (IP) is a condition that may affect several organs, including the skin, teeth, eyes, and  central nervous system (CNS) . The aim of the research is to present a descriptive study of neurological manifestations in 25 cases of IP, and to determine the type and severity of these anomalies. Methods: Retrospective analysis was performed on the clinical data of 25 children with IP, including clinical manifestations (cutaneous, hair, nail, teeth and ophthalmologic abnormalities), age of onset, family history, psychomotor development, CNS types of anomalies, radiological [ultrasonography, computed tomography and magnetic resonance (MRI)] and neurophysiological findings [electroencephalogram (EEG) ]. Results: The average age of onset was 8.1 months; CNS anomalies were diagnosed for 32%: the most common were developmental delay (20%), mental retardation (20%), microcephaly (16%), and epilepsy (12%): 1 West Syndrome who evolved to partial epilepsy, and 2 partial epilepsies. One of the patients presented muscle hypotonia, muscle weakness with no reflexes, neurogenic damage in electromyography, Spinal Muscle Atrophy molecular genetic test negative. EEG revealed characteristic findings in 20% patients (2 generalized EEG abnormalities, 2 focal EEG abnormalities and 1 hypsarrhythmia). MRI showed 8% patients with white matter hyperintensities and 4% polymicrogyria. Conclusion: We found similar number of neurological features than literature has reported. Neurological impairments were the most severe consequences. A wide spectrum of neurological presentations was found. Due to this, and because neurological anomalies usually occur from the neonatal through the early infantile period, fast referral to pediatric neurologists should be routinely considered