Type IV Ehlers-Danlos syndrome (EDS IV), unlike other forms of EDS, appears to be associated with a high incidence of pregnancy complications. In a group of fourteen families 20 women with EDS IV were identified. The diagnosis was confirmed in at least 1 member of each family by in-vitro measurement of type III collagen production by dermal fibroblasts, and all affected subjects produced lower levels of the protein than controls. Of the 20 women identified, 10 had been pregnant, and 5 had died from pregnancy-related complications. The overall risk of death in each pregnancy in this group was 25%. The complications of pregnancy included rupture of bowel, aorta, vena cava, or uterus, vaginal laceration; and post-partum uterine haemorrhage. The severity and frequency of the complications in this type of EDS warrant careful counselling before pregnancy and care of all pregnant patients in a high-risk facility.