A case of the rare fetal brain tumor is presented. This initally was observed as bleeding and ventriculomegaly and it was diagnosed at 28th week gestation by 2D, 3D and TUI ultrasound. The patient remained under the perinatal care until the end of pregnancy: cesarean section was performed at 37th week of gestation. Despite neurosurgery, the baby died on the 3rd day of postnatal life. On the basis of histopathological examination the diagnosis was established such as highly malignant tumor with focal ependymal and neuronal differentation that expands the current histopathology tumors classification.