Objectives. A review of the literature reports neonatal thrombosis and antiphospholipid antibodies cases through a retrospective study that focuses on the pathogenesis and main clinical and laboratory manifestations of this disease. Methods. The case reports were selected from PubMed. The keywords used to search were neonatal, antiphospholipid syndrome, thrombosis, and antiphospholipid antibodies. References that were published from 1987 to 2013 were reviewed. Results. Twenty-one cases of neonatal thrombosis and antiphospholipid antibodies were identified. Ten children were born preterm (before 37 weeks). Arterial involvement (17/21) was predominant, of which stroke (12/17) was the most prevalent clinical manifestation. Anti-cardiolipin antibodies were predominant (13/21) in the antiphospholipid antibody profiles. Treatments were based on the use of symptomatics such as antiepileptics (8/21), and 6/21 patients received heparin. There were 4 deaths (4/21); otherwise, the children recovered well, especially the neonates who suffered from strokes (9/12). Conclusion. Neonatal thrombosis and antiphospholipid antibodies are rare. The development of thrombotic manifestations in neonates seems not to be associated exclusively with the aPL, but their etiology may be linked to pre- and perinatal events. We noted good therapeutic responses, especially in stroke patients, who presented with favorable outcomes in 82% of the cases.