Anti-phospholipid syndrome (APS) is a potentially life-threatening autoimmune condition characterized by the presence of anti-phospholipid antibodies (aPL) giving rise to increased hypercoagulability, which induces venous or arterial thrombotic events at whatever age and recurrent fetal loss in the fertile age. Antigens that are targeted by aPL include cardiolipin and beta2-glycoprotein I. Primary APS is defined in the absence of an underlying disease, while secondary APS is observed in the context of another established pathological condition. APS has a wide variety of clinical signs and serological characteristics. This paper describes the current approaches towards diagnosis, therapeutic modalities and secondary prevention applied to children.