Objectives: To offer a comprehensive review of the literature on Congenital heart block (CHB) in newborns born to women with systemic lupus erythematosus (SLE). Methods: A comprehensive computerized search of pertinent databases was conducted in order to find studies that satisfied the inclusion requirements. To find pertinent information, a thorough search of PubMed, SCOPUS, Science Direct, Cochrane and Web of Science was conducted. Results: Our data included eight trials with 23,967 women diagnosed with SLE. The prevalence of CHB in neonates born to mothers diagnosed with SLE ranged from 0% to 4.2% with a total prevalence of 41 (0.2%). Congenital and acquired heart conditions in children, such as structural and arrhythmic, are linked to maternal SLE. These adverse events were mainly associated with the disease activity. Anti-Ro/SSA antibodies have been related to both CHB and neonatal lupus through transplacental transfer. Conclusion: We demonstrated a low prevalence of CHD in neonates of mothers with SLE. However, the presence of maternal autoantibodies is the primary cause of neonatal CHB, which continues to be a serious pregnancy problem for moms with systemic lupus erythematosus. Although improvements in newborn care and prenatal monitoring have led to better results, problems still exist with early discovery, efficient treatment, and long-term follow-up.