Incontinentia Pigmenti is an X-linked dominant neurocutaneous disorder with 
central nervous system manifestations in 30% of cases, including seizures and 
mental retardation. Ischemic or hemorrhagic cerebrovascular accidents have been 
reported rarely in incontinentia pigmenti. Chart review and literature search 
was performed following identification of the index case. We describe a patient 
with incontinentia pigmenti who developed bilateral cerebrovascular accidents in 
the neonatal period, with resultant severe neurologic sequelae. This is the 
second reported case of bilateral cerebrovascular accidents in a patient with 
incontinentia pigmenti. This finding may be secondary to cerebrovascular 
anomalies, similar to those observed in the retina. Recognition of 
cerebrovascular accidents as a complication of incontinentia pigmenti will 
hopefully lead to earlier recognition and treatment.