Fetal intra-abdominal umbilical vein varix (FIUVV) is a rare pathology and suggests an enlargement of the umbilical vein. Prenatal diagnosis is done through meticulous ultrasound imaging. Management variables are the diameter of varix, presence or absence of turbulent flow, and fetal anomalies. Color and power Doppler ultrasound helps in diagnosis and follow-up and has a critical role in decision-making for intervention. Clinical guidelines for management are not clear. The adverse fetal outcome is usually associated with multiple malformations, turbulent flow, and thrombotic varix. This case study presents the prenatal diagnosis, obstetric management, and neonatal development of a fetus, diagnosed with FIUVV along with a brief review of the literature.