Ehlers-Danlos syndrome is a complex hereditary connective tissue disorder that 
is characterized by abnormalities of the skin and joints and visceral and 
neurological manifestations. At present, at least 11 forms are recognized on the 
basis of their clinical characteristics, methods of transmission, and 
biochemical defect. The neurologic manifestations include cerebrovascular 
disease, peripheral neuropathy, plexopathy, periventricular subependymal 
heterotopias, and epilepsy. Previously, 2 females were reported to be affected 
with subependimal periventricular heterotopias and Ehlers-Danlos syndrome type 
1. The authors report a new case of a 12-year-old girl with similar clinical and 
neuroradiological features.