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Antalya Eğitim ve Araştırma Hastanesi, Beyin ve Sinir Cerrahisi Kliniği, Antalya, Türkiye Congenital intracranial tumors constitute a small percentage of childhood brain tumors. The most common type of these tumors is teratomas. They represent 0.5% of all pediatric intracranial tumors. Most of the teratomas tend to locate at midline structures, preferably sellar and pineal regions. We report a 1-month-old female baby with a poor prenatal evaluation. She came to our attention with a history of irritability, difficulty feeding and “sunset view” at gaze. Further evaluation with magnetic resonance imaging (MRI) diagnosed a large tumor, filling both lateral ventricles. She underwent surgery with right frontal craniotomy. A large grayish-white lobulated vascular mass was removed totally. Histopathological examination revealed an immature teratoma. The prognosis of congenital teratomas is usually poor because these lesions are extensive at the time of diagnosis. Detailed prenatal evaluation of all pregnancies with ultrasonography is important in the early diagnosis. Widespread use of fetal MRI in the near future will provide earlier diagnosis and more insight into the pathogenesis of these rare, and, yet, challenging tumors. Anahtar Kelimeler : Lateral ventricle, Surgery, Teratoma