Purpose <p>Primary intracranial non-germinomatous germ cell tumors, including teratomas, are rare in pediatrics and often pose diagnostic and therapeutic challenges due to their heterogeneous origin and histopathology. This case report aims to contribute to the literature by presenting a rare giant intracranial mature teratoma in a pediatric patient, highlighting its clinical presentation, radiological features, surgical management, and outcomes.</p> Methods <p>We retrospectively reviewed the clinical, radiological, intraoperative, and histopathological data of a 4-year-old boy who presented with imbalance, gait disturbance, and lower extremity weakness. Preoperative serum tumor markers and neuroimaging (MRI and CT) were analyzed, followed by surgical resection and postoperative follow-up.</p> Results <p>Imaging revealed a large heterogeneous tumor extending from the lateral and third ventricles to the cerebral hemispheres, characterized by cystic, fatty, and calcified components suggestive of teratoma. Serum AFP and β-HCG were normal. Complete tumor resection was achieved via a transcortical intraventricular approach. Histopathology confirmed a mature teratoma. Postoperatively, the patient exhibited transient motor weakness and decreased verbal response but returned to baseline within 1 year without neurological sequelae.</p> Conclusion <p>Intracranial mature teratomas, although rare, can be successfully managed with complete surgical excision. Accurate radiological and pathological evaluation is crucial for diagnosis and treatment planning. Complete resection minimizes recurrence and complications, leading to favorable neurological outcomes in pediatric patients.</p>