Fetal ventriculomegaly refers to ventricular enlargement that is diagnosed prenatally. It is one of the most common fetal anomalies. The diagnosis is made by ultrasound when the arterial diameter of the ventricle is more than 10 mm. Once it is diagnosed, further evaluation by detailed ultrasound, fetal MRI, and genetic studies is required. Prenatal surgical management of fetal ventriculomegaly is still limited and associated with high risks. Postnatal management is similar to the treatment of other types of hydrocephalus. Fetal ventriculomegaly is a heterogeneous condition with various etiologies and a wide spectrum of neurodevelopmental outcomes. The outcomes depend mainly on the severity of ventriculomegaly and associated structural abnormalities. This article aims to review the literature about various aspects of fetal ventriculomegaly.