Background <p>To evaluate pregnancy and early neurodevelopmental outcomes in fetuses with prenatally diagnosed ventriculomegaly (VM) in a large single-center retrospective cohort (2011–2024), and to determine the independent predictive value of VM severity, isolation status, laterality, and intrauterine evolution for adverse neurodevelopmental outcomes among term survivors.</p> Methods <p>This retrospective cohort study included fetuses diagnosed with VM at West China Second University Hospital from 2011 to 2024. VM was defined as a lateral ventricular width ≥ 10&#xa0;mm measured on standardized axial trans-thalamo-ventricular planes and categorized as mild (10–12&#xa0;mm), moderate (12.1–15&#xa0;mm), or severe (&gt; 15&#xa0;mm) per SMFM/ISUOG guidelines. Pregnancy and neonatal outcomes were extracted from medical records. Neurodevelopmental outcomes in term liveborn infants were assessed through structured telephone interviews and clinical documentation; for children born between 2019 and 2024, systematic follow-up at 2–3 years of age was implemented, with Gesell Developmental Schedule assessments performed when developmental concerns were identified. For those born between 2011 and 2018, outcomes were based on parental reports and available medical records. Logistic regression models were used to estimate adjusted associations.</p> Results <p>VM was identified in 983 of 154,436 deliveries (0.64%). Live birth rates were 94.8% for mild, 85.9% for moderate, and 24.6% for severe VM. Among 654 term survivors, 39 (5.96%) exhibited abnormal neurodevelopment. In univariable analyses, greater VM severity, bilateral VM, variation of ventricular width, and abnormal postnatal cranial ultrasound findings were significantly associated with adverse neurodevelopment. After adjustment, only ventricular severity remained independently predictive, with adjusted odds ratios of 2.77 (95% CI, 1.31–5.85) for moderate and 7.57 (95% CI, 1.34–42.61) for severe VM compared with mild VM. Non-isolated VM showed a nonsignificant trend toward increased risk (OR 1.64; 95% CI, 0.65–4.14).</p> Conclusions <p>Ventricular severity was the only prenatal factor that remained independently associated with adverse neurodevelopmental outcomes in this cohort, whereas associations with laterality, isolation status, and postnatal imaging findings attenuated after adjustment. Through a unified diagnostic pathway incorporating systematic neurosonography, selective fetal magnetic resonance imaging, and chromosomal microarray testing, this large Asian cohort adds real-world data supporting severity-based risk stratification and illustrating the potential value of multimodal prenatal evaluation for perinatal counseling and management. Given the retrospective design and non-standardized follow-up, however, these associations should be interpreted with caution.</p> Trial registration <p>Not applicable.</p>