The objective of this paper was to review temporal bone chondroblastomas in regard to their presentation, radiographic findings, histopathology, and treatment. A case report of a 38-year-old man who presented with the left-sided hearing impairment and temporal swelling was reviewed. A CT scan revealed an osteolytic lobulated expansile mass. MRI depicted two cystic components with fluid-fluid level and enhanced solid mass. Immunohistochemical study of S-100 was performed using avidinbiotin-complex method. The tumor was totally removed, with eroded squamous bone and temporal muscle, via the left zygomatic-extended middle fossa approach. The pathology of the tumor showed that the tumor cell was spindle-shaped, along with multinucleated giant cells. These cells had oval to polygonal nuclei; some cells showed grooved nuclei. Intercelluar calcification and hemorrhagic components were also observed in the tumor. Tumor cells were strongly positive for S-100 protein. Temporal bone chondroblastomas are extremely rare osseous tumors with only 45 cases previously reported in the published literature. They may be confused with more common lesions seen in the temporal bone. Diagnostic radiology, including CT and/or MRI, as well as immunohistochemical staining with S-100 protein, may assist in making the diagnosis. Treatment is complete surgical excision with preservation of vital neurovascular structures.