The authors describe an infant with abnormal development of the cerebral venous system associated with in utero ventriculomegaly and perinatal hemorrhage. When the patient was 6 weeks of age progressive ventriculomegaly followed by cerebrospinal fluid diversion resulted in tumefaction of the dural sinuses, intravascular thrombosis, and a coagulopathy. Oral anticoagulation therapy safely reversed the coagulopathy.
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