A rare case of pregnancy in a patient with the syndrome of Klippel-Trenaunay-Weber is described. The arterio-venous anomalies in this patient originally occurred in her right leg, but in the course of her first pregnancy she also developed circumscript angiomatosis at the left and right side of the uterus. Her pregnancy was uneventful. However, because of prominent vascular changes in the cervix and lower uterine segment, there appeared to be a cephalo-pelvic discongruency. For this reason a Cesarean section was performed at term. Postoperatively, during heparinization, she had signs of abdominal bleeding, which could be controlled conservatively. There were no signs of intravascular coagulation or cardiac decompensation. A review of the scarce literature on diffuse uterine angiomatosis and angiomatosis occurring as a result of the syndrome of Klippel-Trenaunay-Weber is given. The clinical course and the risks of a pregnancy with this condition are discussed. It is concluded that the angiomatosis based on the syndrome of Klippel-Trenaunay-Weber is less hazardous than a diffuse angiomatosis of the uterus without this disease, the main risk being diffuse intravascular coagulation at or after delivery.