Abstract
A 10-year-old girl with features of Klippel Trenaunay Syndrome developed a large left frontoparietal subdural hemorrhage. CT angiography and cerebral angiography identified prominent subependymal veins and deep venous system predominantly in the left cerebral hemisphere in association with a dilated left vein of Labbe and hypoplastic superior sagittal sinus. No arteriovenous malformation or fistula was identified. Intracranial hemorrhage in Klippel Trenaunay Syndrome is extremely rare and usually traced to an underlying arteriovenous malformation or fistula. To the best of our knowledge neither a venous source of intracranial bleed nor subdural hemorrhage in Klippel Trenaunay Syndrome has been described in the English literature.