The patient is a 17 month old girl admitted to the Korea General Hospital with histories of irritability, walking disturbance and right sided weakness. On admission, she had seizure followed by coma. Skull x-ray showed widely separated all cranial sutures. A left carotid angiogram revealed a space occupying lesion in the left frontotemporoparietal region with shifting of the midline to the right. At operation, a darkish hemorrhagic tumor with relative clear demarcation was explored after evacuation of massive intracerebral hematoma. The tumor was extended to the midline. The main mass was removed with difficulty. Additional fragments of the necrotic tumor tissue infiltrated in the surrounding structures were also removed. It was not directly connected to or have invaded the ventricular wall. On microscopic examination, the tumor was largely made up of solid sheets and cords of round or ovoid cells with darkly stained nuclei. In areas, typical ependymal rosettes as well as pseudorosettes, gliomatous features and occasional formation of tubule are presented. We think the tumor represents those rare tumors called ependymoblastoma of Rubinstein.