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Fetal intra-abdominal umbilical vein varix is a rare condition characterized by focal dilatation of the umbilical vein of the fetus. We report a case of fetal intra-abdominal umbilical vein varix detected at 29 weeks’ gestation. To avoid fetal death, several follow up sonographic examinations were performed and there was no thrombus within the fetal intra-abdominal umbilical vein varix. After Cesarean delivery, however, dilated portal vein with large thrombus connected to umbilical vein was confirmed on abdominal ultrasound, MRI, CT-angiography and operation for resection of umbilical vein anomaly was performed.