Objective: Congenital intracranial tumors are rare and only account for 0.5–1.5% of all pediatric brain tumors. Teratoma is the most frequently encountered intracranial tumor at birth. Massive congenital intracranial teratoma is an extremely rare neoplasm with a poor prognosis. They grow rapidly and cause extensive destruction in the brain. Herein we report a massive intracranial teratoma causing skull rupture. Case Report: A fetus with a congenital intracranial teratoma presenting with a disproportionately enlarged head at 25 weeks of gestation is presented. Since it was the first admission of the mother to a medical expert for a prenatal examination, there was noprevious follow-up data. Prenatal ultrasonography demonstrated a huge, heterogeneous intracranial mass, and midline structures and ventricles could not be observed. No heartbeat was detected. Autopsy was perforated, and histopathologic examination of the samples taken from the intracranial mass revealed an immature teratoma. Conclusion: Although congenital intracranial teratomas are rare, they may reach enormous sizes. Regular follow-up of the fetus may lead to early diagnosis of immature intracranial teratomas and prevent the mother from having further complications either due to intrauterine fetal death orpsychological trauma of giving birth to a heavily malformed baby.

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