BACKGROUND: As in most other autoimmune diseases, Sjögren's syndrome is seen predominantly in women. Since the peak age is around the late reproductive and early postmenopausal period, the obstetric aspect has not been well studied. CASE: A 28-years-old woman, pregnant for 22 weeks and 5 days, was admitted with worsening general status, skin lesions, arthralgias, and oral and ocular symptoms typical of Sjögren's syndrome. She underwent hemodialysis for renal insufficiency. To prevent autoantibody formation, progression of the disease, therapy with methyl prednisolone, 100 mg/d intravenously; cyclophosphamide, 500 mg/month in a single intravenous application; hemodialysis 3 times a week; and plasmapheresis 7 times was instituted. An 1,100-g, male infant at 27 weeks and 5 days was delivered by cesarean section because of premature preterm rupture of membranes and severe late decelerations on cardiotocography. The infant was discharged from the neonatal intensive care unit after 30 days, weighing 1,800 g. Postnatal echocardiographic examination of the infant revealed neither cardiac malformations nor arrhythmias. CONCLUSION: Since the presence of autoantibodies against SS-A and SS-B are reported to accompany congenital heart block, the primary goal of therapy should be preventing this untoward effect of the disease. Close monitoring during pregnancy is mandatory to detect preeclampsia, intrauterine growth retardation and preterm labor.