A 31-year-old woman with a large placental tumor underwent a caesarean section. After delivery, the lesion, detected by ultrasound examination, was found to originate from the umbilical cord. A live female infant with cyanosis, hypotonia and diffuse edema was delivered. A review of the literature, which revealed 31 cases of umbilical cord hemangioma, showed that this tumor has a polymorphous presentation. Some fetuses and infants died from various causes, indicating that a close follow-up is necessary in these pregnancies