Thrombosis risk is multifactorial, with interaction of hereditary risk factors and acquired environmental and clinical conditions. Newborns are at particular risk for thrombotic emergencies secondary to the unique properties of their hemostatic system, influences of the maternal-fetal environment, and perinatal complications and interventions. Thrombotic complications range from arterial and venous catheter thrombosis to purpura fulminans. Prompt identification and appropriate management of thrombotic emergencies is critical in avoiding limb-, organ-, and life-threatening complications. Treatment strategies have been extrapolated from adult literature but clinical experience from small-scale neonatal studies has resulted in therapeutic guidelines, which should be individualized for each neonate, taking into consideration age and clinical status.