Objective: Congenital heart block (CHB) is a rare but life-threatening disorder. More than half of CHB cases are associated with matal autoimmune, which are termed as autoimmune-associated CHB. This review summarized the recent research findings in understanding autoimmune-associated CHB, discussed the current diagnostic approaches and management strategies, and summarized the problems and future directions for this disorder. Data Sources: We retrieved the articles published in English from the PubMed database up to January 2017, using the keywords includingAutoimmune-associated, Autoimmune-mediated, and Congenital heart block. Study Selection: Articles about autoimmune-associated CHB were obtained and reviewed. Results: Observational studies consistently reported that transplacental matal antibodies might recognize fetal or neonatal antigens in various tissues and result in immunological damages, but the molecular mechanisms underlying CHB pathogenesis still need illuminated. Multiple factors were involved in the process of atrioventricular block development and progression. While several susceptibility genes had been successfully defined, how these genes and their protein interact and impact each other remains to be explored. With currently available diagnostic tools, fetal ultrasound cardiography, and fetal magnetocardiography, most of CHB could be successfully diagnosed and comprehensively evaluated prenatally. The efficacy of current approaches for preventing the progression and recurrence of CHB and other autoimmune-mediated damages was still controversial. Conclusions: This review highlighted the relationships between autoimmune injuries and CHB and strengthened the importance of perinatal management and therapy for autoimmune-associated CHB.