Congenital intracranial tumors as a group are quite rare, representing only 0.5% to 1.5% of all pediatric brain neoplasms, of which most were congenital intracranial teratomas.[4–8] Prenatal ultrasonography during the whole pregnancy is of particular importance for screening fetal central nervous system tumors. However, few examples of fetal intracranial mixed neuronal-glial tumors have been described by imaging and fewer cases have been confirmed by histopathological examination. Our case contributes to the limited literature focused on the imaging (ultrasonography and MRI), pathological and genetic discoveries of intracranial mixed neuronal-glial tumor in the prenatal period. Searching through the literatures, we only noticed that Chung et al reported a congenital gangliocytoma in 1998 which fell into this category of intracranial mixed neuronal-glial tumor. Its ultrasound features contained both cystic and solid components, located suprasellar and caused marked displacement of the circle of Willis.