Incidence of venous thromboembolism (VTE) in children is rapidly rising. Frequent use of central venous lines (CVLs) in children with a variety of disease processes has contributed to increased incidence of VTE. In addition, increased detection of VTE has been possible due to the availability of sensitive imaging studies such as Color Duplex/Doppler ultrasound, lineogram and venogram. Heightened awareness of congenital prethrombotic disorders may help identify children at higher risk of thrombosis. Anticoagulation with heparin, low molecular weight heparin (LMWH) and oral anticoagulant such as warfarin are used for the treatment of VTE. Thrombolytic therapy may be indicated in some cases. Clinical presentation, management and pathogenesis including role of congenital prethrombotic disorders in pediatric VTE are discussed.