Background: Congenital disseminated intravascular coagulation (DIC) has been reported in a variety of clinical settings, but to our knowledge, it has never been documented before birth. We describe prenatal identification of an umbilical cord vascular tumor and echogenic foci in the fetal liver leading to the diagnosis of fetal DIC by funipuncture.Case: A large vascular tumor of the umbilical cord was identified using prenatal sonography. At 31 weeks' gestation, echogenic areas, which were thought to represent infarcts or thrombi, were seen in the liver. When funipuncture revealed florid fetal DIC, delivery was accomplished by cesarean to prevent intracranial bleeding. Pathologic examination of the umbilical cord tumor showed it to be a large arteriovenous malformation.Conclusion: Arteriovenous malformations may cause fetal coagulopathy. This case illustrates that funipuncture may be used to diagnose this condition and that timely cesarean delivery may contribute to a favorable outcome.