Antiphospholipid syndrome (APS) is characterized by venous or arterial thrombosis and/or fetal losses, in the presence of one or more types of antiphospholipid antibodies (aPL) (Anitcardiolipin antibodies (ACA), lupus anticoagulant (LA) or anti beta 2-glycoprotein I (β2GPI). Information about APS in juvenile patients is limited and reports showed some differences between the clinical and serological features in adults and juvenile APS. There are limited data on the incidence and prevalence of Primary APS (PAPS) and Secondary APS (SAPS). Thrombotic events are the typical manifestation of APS but hematologic and neurologic manifestations have been described with different frequencies.