High-resolution prenatal ultrasound can allow for early detection and monitoring of many fetal anomalies, including those involving the globe and orbit. The authors present a case of congenital axial high myopia that was diagnosed at 33 gestational weeks and monitored by prenatal ultrasonography. Some systemic abnormalities that can be associated with congenital high myopia are reviewed. High ammetropias, anisometropia, and either axial myopia or axial hyperopia can be detected in utero and give this early detection a greater chance of treating and reversing the devastating effects of amblyopia.