This study aimed to report unusual location and imaging findings of an intracranial chondrosarcoma in a 7-year-old child. Chondrosarcoma is a malignant tumor arising from the chondrocytes that remain at the synchondroses of basilar skull bones. It is difficult to differentiate chondrosarcoma from chordoma because they are similar in appearance. The prognosis and survival rate of patients depend on the pathological type and degree of resection of tumors. In this study, the patient was in a comatose state on admission. The computed tomography and magnetic resonance imaging of the head revealed a mass lesion extending from the left thalamus to the left cerebellar peduncle, and the imaging findings looked like a granulomatous sphere from fungal infection. The mass lesion was completely resected, and chondrosarcoma was diagnosed after pathological examinations. This tumor was present in an unusual location, compared with most chondrosarcoma cases, with atypical imaging findings, hindering the diagnosis of this intracranial chondrosarcoma case on admission. Also, this study reviewed the incidence of intracranial chondrosarcoma in children since 1963 and analyzed the treatment modalities related to the prognosis. The study showed that the prognosis and survival rate of patients with chondrosarcoma depended on the pathological type and treatment modalities.