Familial incontinentia pigmenti (IP) (OMIM #308300) is a rare genetic disorder 
which segregates in an X-linked dominant way. The female-to-male ratio ranges 
from 20 to 37 : 1. In affected females IP causes highly variable abnormalities 
of the skin, hair, nails, teeth, eyes, and central nervous system (CNS). 
Cardiovascular anomalies, cerebral infarction, and immune dysfunction are rare 
complications of IP. The pathogenesis of cerebral changes in IP remains elusive. 
We report the case of two IP-affected sisters who presented in each case with 
neonatal seizures on the fifth day of life. Via cranial magnetic resonance 
tomographic imaging (MRI) different types of lesions in both hemispheres were 
demonstrable in both patients. To date the pathogenetic mechanisms for the 
cerebral lesions are not fully understood. However, multiple microscopic 
infarcts could serve as a possible explanation. The clinical course and the 
neurological development of the older child are favorable and so far the younger 
sibling appears to be developing normally, which is uncommon for patients with 
early onset of neurological symptoms. Symptomatic seizures in IP are an 
important differential diagnosis in benign non-familial and familial neonatal 
seizures.