Lupus anticoagulants (LAs) are a heterogeneous class of immunoglobulins that specifically target the epitopes of the negatively charged phospholipid binding protein of cell membrane, prothrombin, and beta2-glycoprotein I (beta2-GPI) which inhibit phospholipid-dependent coagulation. LAs, an acquired clotting factor inhibitor, poses unique diagnostic and clinical challenges in pediatric patients. This review highlights recent advancements in understanding pediatric LAs, focusing on their clinical significance, diagnostic methodologies, and therapeutic approaches. The association of LAs with autoimmune diseases, such as systemic lupus erythematosus and antiphospholipid syndrome, underlines their clinical relevance. Pediatric LAs may lead to thrombotic or hemorrhagic events, significantly impacting a child's quality of life. Diagnostic approaches include comprehensive clinical evaluations and laboratory testing, with assays like activated partial thromboplastin time (aPTT) and dilute Russell viper venom time (dRVVT) playing critical roles. Despite these tools, challenges remain due to the variability in test sensitivity and specificity, transient LAs positivity, and the potential for misdiagnosis. This work underscores the importance of multidisciplinary collaboration among pediatric hematologists, neonatologists, and intensivists to ensure accurate diagnosis and effective management. By addressing gaps in pediatric LAs research and highlighting emerging diagnostic and therapeutic strategies, this study aims to improve outcomes and quality of care for affected children.