Introduction Congenital complete atrioventricular (AV) block manifests in approximately 1 in 20,000 live births and poses significant risks of mortality and morbidity both prenatally and postnatally. Its etiology can stem from either immune or non-immune mediated pathways. Immune-mediated AV block is a complex condition often linked to the transplacental transmission of maternal autoantibodies, particularly anti-Ro/SSA and/or anti-La/SSB antibodies. Implanting permanent pacemakers in neonates with congenital complete AV block presents technical challenges due to their small size, the presence of concurrent structural heart anomalies, and the rapid growth rate typical of infants. Case description We encountered five instances of congenital complete atrioventricular (AV) block in neonates exhibiting persistent low heart rate post-birth. Among these cases, two patients had mothers with a history of autoimmune disease, specifically Sjogren's syndrome with positive Ro antibodies and receiving methylprednisolone, while the remaining three cases had no history of autoimmune disease. Additionally, one case presented with a congenital anomaly, namely anorectal malformation, and three cases were complicated by sepsis during hospitalization. Diagnosis was established perinatally, with intervention involving immediate permanent pacemaker implantation after birth in two cases, and in the remaining three cases, permanent pacemaker implantation occurred within the subsequent days. Encouragingly, all five cases exhibited favorable outcomes without complications. Conclusion Congenital complete AV block represents a rare yet critical condition necessitating prompt recognition. Early diagnosis and treatment, particularly through permanent pacemaker implantation, play pivotal roles in mitigating morbidity and mortality rates associated with this condition, ultimately leading to improved patient outcomes.