This report presents an extremely rare case of complicated fetal esophageal atresia (EA) with tracheoesophageal fistula (TEF) and interrupted inferior vena cava (IVC), successfully diagnosed through prenatal ultrasonography and treated with surgical intervention. A 35-year-old pregnant woman was referred to our center for a routine prenatal ultrasound. During the examination, several significant abnormalities were identified, including an interrupted IVC associated with a dilated azygos vein, an upper neck pouch sign in the thorax, and polyhydramnios. These findings raised suspicion for EA with TEF and interrupted IVC. The infant was delivered at 39 weeks of gestation and promptly underwent surgical repair of the identified anomalies. Post-operative recovery was successful, and the baby has continued to thrive during the 21 months of follow-up. This case highlights the critical role of prenatal ultrasonography in diagnosing complex congenital conditions, allowing for optimized labor care and informed postpartum treatment planning. Early detection of such anomalies is crucial, as it facilitates appropriate neonatal management and can significantly improve outcomes for affected infants. This report underscores the importance of advanced imaging techniques in prenatal diagnosis, emphasizing how timely intervention can lead to successful treatment outcomes in cases of severe congenital abnormalities.