The incidence of thrombotic events is higher in neonates compared to children of other age groups. Perinatal and maternal risk factors, as well as the developing neonatal hemostatic system, are contributing factors in the higher rate of thrombosis in this pediatric population. This review summarizes the available literature to date and gives an updated overview of the systemic venous thrombosis in neonates including clinical presentation, risk factors, recommended treatment modalities and associated outcomes.