Glioblastoma (GBM) of the spinal cord represents a rare entity in children and account for less than 1% of all central nervous system (CNS) cancers. Their biology, localization, and controversial treatment options have been discussed in a few pediatric cases. Here, we report a case of primary spinal cord glioblastoma in a 5-year-old girl having the particularity to be extended to the brainstem. This tumor has been revealed by torticollis and bilateral brachial paresis. The patient underwent subtotal resection; unfortunately, she died in reanimation 1 week later by severe pneumopathy. To the best of our knowledge, this is the first case in the literature reporting this particular localization in a child. Beyond their dismal prognosis, we discuss the rarity of the disease and describe the peculiar characteristics, management, and prognosis of this rare tumor in pediatric oncology. This case appears to be unusual for both the histological type and the extension to brain stern.