Antiphospholipid syndrome (APS) is rare in children and characterized by systemic thrombosis with positive antiphospholipid antibodies (aPL), such as lupus anticoagulant (LA) or anticardiolipin antibodies (aCL).[2] APS can occur secondary to other diseases, such as systemic lupus erythematosus (SLE); however, this case is a primary APS occurring independently. This evidence concerns the gene ACLY and autoimmune polyendocrinopathy.