The perinatal findings of a pregnancy complicated by an umbilical cord abnormality associated with a small omphalocele are presented. The lesion was first observed at 18 weeks of gestation and was associated with elevated maternal serum alpha-fetoprotein. Serial ultrasound examinations showed major changes in the cord appearances and an associated small omphalocele was identified during the third trimester. Colour flow imaging showed no blood flow within the lesion and no obstruction nor involvement of the main umbilical vessels. Postnatal investigations demonstrated focal myxoid degeneration of the cord, a small omphalocele and no other fetal abnormalities.