We describe a successful management of a pregnancy in a woman with type IV Ehlers-Danlos syndrome that included an elective caesarean section and tubal ligation at 32 weeks' gestation. We identified 26 women (including ours) who had been pregnant with well-documented characteristics of type IV Ehlers-Danlos syndrome in the literature. These 26 women had 50 pregnancies. Ten (38.5%) died during pregnancy or in the immediate postpartum period. The maternal mortality rate per pregnancy in type IV Ehlers-Danlos syndrome was therefore calculated to be 20.0%. Pregnancy, continuing pregnancy, or subsequent pregnancy are all contraindicated in patients with type IV Ehlers-Danlos syndrome. Termination of pregnancy before 16 weeks' gestation should be strongly advised. If the patient proceeds with pregnancy, restriction of physical activity at the beginning of third trimester, frequent evaluation of the patient, and an elective caesarean delivery at 32 weeks' gestation after an appropriate antenatal steroid therapy should be advised.