Introduction: Fetal intracranial tumors represent only 10% of all antenatal tumors; teratoma being the most common accounting for approximately half of all reported cases followed by astrocytoma, lipoma, choroid plexus papilloma, craniopharyngioma, and primitive neuroectodermal tumor. Usually these tumors clinically manifest in the third trimester of pregnancy, which otherwise nowadays often recognized earlier in pregnancy by ultrasonography and magnetic resonance imaging. Majority of fetal intracranial tumors are supratentorial, however in larger lesions imaging or even autopsy is frequently unable to determine the origin.
Case Report: Here we are presenting a rare case of large fetal intracranial teratoma with which the patient presented in the third trimester with fatal outcome.
Conclusion: Progress in technology has contributed to early diagnosis of congenital CNS tumors, but the same is not observed with fetal surgery, perhaps because the prognosis of fetal brain tumors remains poor. It is critical to have the most precise information regarding the tumoral nature and the extension of the lesion. Fetal medicine centers should be composed of a multidisciplinary team acting together to provide better assistance for fetuses with congenital CNS tumors and to develop new methods of treatment.

Keywords: Antenatal intracranial tumors, Immature teratoma