Rationale:Patients with situs inversus totalis (SIT) and complete atrioventricular (AV) block are extremely rare, and only few cases have been reported up to now. Due to the rarity of such condition and its anatomical complexity, we reported this case as a reference for obstetricians, providing valuable insights into potential clinical treatment.Patient concerns:We reported a case of 30-year-old patient with complete AV block, and her heart rate was only about 45 beats per minute.Diagnoses:The patient was diagnosed with term delivery with SIT and complete AV block.Interventions:A temporary pacemaker (TPM) was implanted before cesarean section (CS) because of complete AV block.Outcomes:CS was performed successfully after the implantation of a TPM. The heart rate remained stable, and the 24-hour blood loss was limited.Lessons:This study presents a complex heart disease case which needed more frequent antenatal examination and restriction of physical activity. To reduce the risk of pregnancy, basic disease needed to be cured before pregnancy. Our findings could provide guidance for future clinical studies.