Ehlers-Danlos syndrome type II is a rare connective tissue disorder with unknown pregnancy-related maternal and fetal morbidity. The course and outcome of pregnancy in a primigravid woman with Ehlers-Danlos syndrome type II is described. At 17 weeks' gestation a prophylactic Shirodkar cerclage was performed without complications. From the 29th week on, decreased blood flow of the umbilical artery was measured, and fetal growth retardation was evident from 34 weeks' gestation on. Pregnancy ended at 41 weeks by spontaneous vaginal delivery of a healthy 2900-g female infant. Previous reports of 16 women with Ehlers-Danlos syndrome type II, who had a total of 24 pregnancies, are reviewed.