Cerebral sinovenous thrombosis (CSVT) is an uncommon condition among paediatric patients involving major sinuses, with a preponderant occurrence in neonates. The clinical presentation is unspecific, either early, within 48h from birth, or later. An early presentation may be accompanied by several comorbidities (respiratory distress, poor tone, fetal distress, asphyxia), whereas a later presentation is more often associated with conventional neurological signs such as seizures, lethargy, apnoea and poor feeding. These differences in clinical presentation render the neuroradiological diagnosis difficult, in particular before the introduction of magnetic resonance imaging. The interest in CSVT is based on the complex pathogenesis, often resulting from a combination of inherited and acquired thrombophilic patterns. In addition, the course of CSVT can be influenced by medical treatment, currently based on the consensus of experts more than on randomised trials.