Abstract: Antiphospholipid syndrome (APS) is consifered as most common acquired hypercoagulation state of autoimmune disorder in children. Besides vascular occlusion, antiphosholipid antibodies have been associated with various nonthrombotic clinical manifestations. Because of children do not suffer from the frequent thrombosis, which is due to the lack of risk factors and because of immature hemostatic and immune system, their treatment is often controversial, consisting of identifying existing risk factors, preventive medicine and the short-term treatment of thrombotic events. Clinical and laboratory characterisation of pediatric patients continues to improve and implies differences between adult and pediatric populations. Nowadays there are few reported cases of new targeted biological treatment of APS by rituximab and plasmapheresis. This article presents an overview of literary knowledge of the antiphospholipid syndrome in children, its diagnosis dependent on clinical and laboratory criteria for heterogeneous clinical manifestations, which is not completely identical to adult population and the treatment of patients with this rare disease...