Dilatation of the fetal umbilical vein is a rare, most commonly isolated finding. Approximately 100 cases have been reported in the literature that describe different management approaches, especially regarding the time of delivery. We present a new case of umbilical vein dilatation diagnosed at 23 weeks’ gestation as an isolated sonographic finding, in a fetus with short umbilical cord, delivered at 38 weeks’ gestation. The clinical and sonographic features as well as the management options of this uncommon condition are shortly discussed.