The widening of the vestibular dimension of lateral ventricles > 10 mm should be considered a symptom rather than a definitive diagnosis. In fact, fetal ventriculomegaly (VM) is a defect with ’multifaceted‘ clinical consequences in the child’s further neurodevelopment. Isolated fetal ventriculomegaly can cause neurological defects ranging from mild neurodevelopmental delay to severe complications in the form of ongoing palliative care to the death of patients at various developmental periods. The spectrum of compilations often depends on the severity of the ventriculomegaly. In the prenatal period, the combined diagnostic tools include the following: ultrasound/MRI and genetic, infectious tests that form the basis of reliable counseling. We hypothesize that advances in the diagnostic process allow the identification of ‘probably’ isolated forms of severe VM (ISVM). The review authors electronically searched MEDLINE, EMBASE, and the Cochrane Library databases, describing the evidence-based validity and option of prenatal decompression for ISVM. The purpose of this review is to present the evolution of diagnostic techniques and views indicating the possibility and limitations of implementing prenatal decompression in severe ISVM. In conclusion, after reviewing the available data, we want to introduce the idea that perinatal centers are close to or have reached the necessary capability, expertise, and competence to perform ISVM decompression procedures. Endoscopic ventriculostomy of the third ventricle (ETV) appears to be promising, as it seems to be associated with minimal perinatal complications and better neurological outcomes for the newborn. However, long-term follow-up results for the neurodevelopment of patients who underwent ETV have not been reported. Looking ahead, randomized trials with the long-term neurodevelopmental follow-up of children who underwent prenatal decompression due to ISVM are needed.