Neurenteric cyst is a very rare developmental anomaly. Prenatal diagnosis of mediastinal neurenteric cysts has been reported rarely. We present a case of neurenteric cyst associated with vertebral anomalies diagnosed by prenatal ultrasonography at 31 weeks of gestation, which was treated successfully in the early neonatal period. In addition, we searched the English literature for all cases of mediastinal neurenteric cyst diagnosed in the prenatal period reported to date. We found that only 17 cases were reported previously. We reviewed the reports of these 17 patients along with our case, and we investigated the prenatal and postnatal diagnosis and treatment approaches and the factors influencing the prognosis. Fetuses with mediastinal neurenteric cysts should be monitored regularly by ultrasonography. Fetuses with no signs of hydrops are more likely to survive with proper neonatal center transfer, regular follow-up, and appropriate postnatal approach. Fetuses with hydrops findings have a high risk of fetal and neonatal death.