Abstract
Congenital brain tumors (CBTs) are extremely rare and account for only 0.5%-1.9% of all pediatric brain tumors. Medulloepithelioma is one of the rare tumors with an incidence of about 1% among all CBTs with a very dismal prognosis and typically diagnosed at the median age of 24 months. The objective is reporting medulloepithelioma presenting in the intrauterine period with very few prior cases being reported in the prenatal period, and to add to the limited existing literature on medulloepithelioma. We present a rare case of medulloepithelioma referred to us in the antenatal period at 27 weeks and subsequently causing intrauterine fetal demise. Prenatal MRI of the fetal brain and postnatal histopathological findings on autopsy were suggestive of intracranial medulloepithelioma.