Hypomelanosis of Ito (HI) is a neurocutaneous syndrome with multisyste
mic involvement, Its most frequent neurological abnormalities are ment
al retardation and seizures. EEG, CT and MRI findings are not characte
ristic enough to be diagnostic. In this report, we describe a patient
with typical cutaneous lesions of HI and an intracranial arteriovenous
malformation (AVM), which has not been previously reported.