Degenerative areas with accumulation of mucoids in Whartons jelly are not uncommon. Such degeneration may occasionally be so pronounced as to cause cyst formation. Mucoid degenerative cyst of the umbilical cord associated with neonatal death has been reported rarely in literature. Recently we experienced a case of neonatal death caused by mucoid degenerative cysts of the umbilical cord. A 26-year-old primigravida woman was referred for ultrasound examination at 37 weeks gestation. Ultrasonography revealed two cystic masses in the umbilical cord which were located weveral centimeters away from the fetal umbilicus and hydramnios. Emergency cesarean section was taken due to late deceleration during progress of labor on the 37 weeks gestation. The woman delivered a live 2290 g male infant with Apgar scores of 2 and 3 at one and five minutes. Soon later, we performed resuscitation due to apnea. But the neonate died at one hour after delivery. Two mucoid degenerative cysts with dimention fo 3.5*2.5Cm and 2*1 Cm were located 2 Cm and 5 Cm away from the neonatal umbilicus. We present this case with brief review of the literatures.