Hemangiomas of the umbilical cord are extremely rare benign vascular tumors, not always detected prenatally. They have been associated with increased alpha-fetoprotein (AFP), hydramnios, congenital anomalies, and increased perinatal mortality. Impaired umbilical circulation has been proposed as the predisposing factor for fetal compromise. We report a case of an antenatally detected umbilical cord hemangioma with one artery crossing the tumor, and we reviewed the literature. Close surveillance with Doppler flow studies of the umbilical vessels were carried out throughout the pregnancy. All indices were normal, except from the intra-tumoral part of the umbilical artery under discussion that showed increasing resistance from 32 weeks onwards. Our review confirmed the reported association with increased AFP and hydramnios. The placental end of the cord was the preferred site of location, and the umbilical artery the commonest vessel of origin. Association with cutaneous vascular malformations, and single umbilical artery were assessed.