Desmoplastic cerebral glioblastoma has been described recently and is a very rare histologic variant of glioblastoma. We report a case of congenital cerebral glioblastoma associated with intense desmoplastic stromal reaction. A male infant was born at 36 gestational weeks by Cesarian section. He had a brain tumor, which was detected by fetal ultrasonography. The tumor was partially resected 2 months after the day of the boy's birth and totally resected when he was one year old. The microscopic features of the tumor were those of glioblastoma, including high cellularity, frequent mitotic figures, vascular endothelial proliferation, and geographic palisading necrosis. The tumor showed an area of intense desmoplasia where tumor cells were surrounded by dense reticulin fibers. The desmoplastic cerebral tumors in children may be a distinct group of brain tumor, and it is important to understand the entity of these tumors which generally seem to be associated with more favorable prognosis compared to other high grade brain tumors.