A 7-year-old girl with a 1-month history of neck pain...A diagnosis of spinal cord glioma was suspected….Immunohistochemistry showed the tumor harbored an H3 K27M mutation...Oral temozolomide was administered for 7-months...After chemoradiotherapy, the muscle strength of right upper limb was improved, and the residual tumor was smaller than before. After an 18-month follow-up, the child's tumor did not progress, and the child's condition was stable and recovered well.