Aim: The purpose of this retrospective study was to examine the association of ventriculomegaly in cases of CNS anomaly and to study the sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) of ventriculomegaly in predicting CNS anomaly. We have also tried to compare the predictive value of ventriculomegaly in predicting CNS anomaly to cardiac axis deviation in predicting cardiac anomaly. Materials and methods: Over a period of 5 years, all cases of CNS anomalies with or without ventriculomegaly with gestational age more than 14 weeks were reviewed for the size of lateral ventricles, other CNS findings, extra-CNS structural anomalies. Ventricle size up to 7.0 mm was considered normal, the size from 7.1 to 10.0 mm was considered as prominent ventricle, from 10.1 to 14.9 mm was considered as mild ventriculomegaly and the size of ≥15 mm was considered severe ventriculomegaly. Results: In total, there were 206 CNS anomalies detected in 165 patients. The commonest CNS anomaly detected was agenesis of corpus callosum (ACC) and partial agenesis of corpus callosum (PACC). Our data show that although ventriculomegaly is a strong marker for suspecting intracranial structural abnormality, anomalies of posterior fossa, PACC, and intracranial cysts may be present with normal-sized ventricles. Conclusion: From our study, we can conclude that ventriculomegaly does not predict the presence of structural CNS abnormality, and a normal size ventricle rules does not rule out the presence of structural CNS anomaly. Ventriculomegaly is not a strong marker in CNS anomaly like cardiac axis deviation in cardiac anomaly.