Neonatal venous sinus thrombosis is a well-recognized, but infrequently diagnosed, cause of neonatal encephalopathy. Previous reports have tended to omit reference to the importance of maternal factors in predisposing the infant to this condition. This report, in which eight patients with neonatal venous sinus thrombosis are presented, will reveal a strong association between pre-eclampsia, prothrombotic disorders, and neonatal venous sinus thrombosis. Contrary to previously published reports, there is a high likelihood of neurodevelopmental residua after this condition.