Neonates are known to have a higher risk of cerebral sinovenous thrombosis than children of other age groups. The exact incidence in neonates remains unknown and is likely to be underestimated, as clinical presentation is nonspecific and diagnosis can only be made when dedicated neuroimaging techniques, including computed tomographic venography or magnetic resonance venography, are performed. Associated intracranial lesions are common and some, such as a unilateral thalamic hemorrhage, should suggest cerebral sinovenous thrombosis as the underlying etiology. Neurodevelopmental outcome is poor in approximately 50% of these infants and is adversely affected by associated parenchymal lesions. Anticoagulation therapy will limit propagation of the clot and possibly the development or enhancement of parenchymal lesions. Multicenter randomized clinical trials are urgently needed to address many of these important issues.