Abstract Over the last decade, pediatric thrombophilia programs have emerged around the world as a new discipline in pediatric hematology. These programs specialize in the diagnosis, prevention and treatment of children with thromboembolic events (TEs) in both the venous and arterial systems. The need for separate pediatric programs has been discussed previously. (J Pediatr Hematol Oncol 1997; 19: 7–22.) The following article will update previous reviews (Hematol Oncol Clin North Am 1998; 12: 1283–1312; Thromb Haemost 1997; 78: 715–725) and will concentrate on three aspects: (1) The risk factors for acquiring TEs; (2) The confirmatory diagnostic tests used in children with TEs; and (3) The different antithrombotic agents used for prevention and treatment. The current knowledge in respect to the above points is only the ”tip of the iceberg”. Well-designed prospective trials are required to establish the contribution of congenital prothrombotic disorders, appropriate diagnostic strategies, and optimal therapy for children with TEs.