Klippel-Trenaunay syndrome (KTS) is a rare, complex congenital vascular malformation. This neurocutaneous syndrome can be associated with brain malformations. We report a case involving Klippel-Trenaunay syndrome and hemimegalencephaly in a 3-year-old child revealed by epileptic encephalopathy. We discuss the clinical features and the contribution of imaging in this association.
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