Objective: We report the case of a premature newborn with congenital atrioventricular block (CAVB) and with situs inversus totalis (SIT), in whom a single-chamber endocardial pacemaker was implanted. Clinical case: Two-day-old female who was born at 35 weeks gestation and with low birth-weight. A 12-lead electrocardiogram showed a ventricular rate of 68 bpm, negative P waves in D1 and aVL and positive in aVR, with atrioventricular block 2x1. Sequential segmental analysis determined situs inversus, while a thoracic-abdominal CT scan confirmed SIT. Because data on low cardiac output were observed in the first 48 hours, and after ruling out causes of BCVB, it was decided to implant a single-chamber pacemaker endocardially. The patient evolved without complications. Conclusion: SIT is a condition that can be associated with BCVB; pacemaker implantation, even in premature infants, can improve the prognosis for these patients.