We report an umbilical cord hemangioma associated with an hydrops fetalis and detected by ultrasonography at 21 week's gestation. Because of the fetal distress, a caesarean section was performed at 28 weeks'. The infant died soon after birth despite resuscitation. The post-mortem examination showed an extensive accumulation of serous fluid in the fetal tissues and the body cavities, and major pulmonary hypoplasia. The umbilical cord contained a grossly fusiform tumor which fulfilled the pathological criteria of an umbilical cord hemangioma. Umbilical cord hemangiomas are very unusual tumors and are rarely associated with a fetal hydrops. This tumor is a cause of increased maternal serum and amniotic fluid alpha-foetoprotein levels. Although it is usually isolated, it is imperative to search for associated malformations or angiomas that may clinically manifest later.