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The objective of this study is to describe the presentation and clinical course of two patients with temporal bone chondroblastoma, and to review the literature on temporal bone chondroblastoma to identify characteristic clinical and radiological presentations, and optimal treatment regimens. MEDLINE literature searches covering the period from 1966 to January 1998, in all languages, were performed as well as a review of the bibliographies of the identified studies. Strict inclusion criteria were upheld, In total 18 studies had patients whose data could be analyzed. From the 18 studies, 34 patients were identified, but only 21 cases met the inclusion criteria. Demographic, clinical presentation, radiological, operative and treatment parameters were analyzed in this cohort of patients. Ninety-five percent of patients were found to have invasion of the middle cranial fossa and 76% were found to have erosion into the superior aspect of the external auditory canal by temporal bone chondroblastoma. The characteristic growth pattern of temporal bone chondroblastoma may result from embryonal or cartilagenous rests entrapped in the tympanosquamous suture line in the middle fossa floor. Temporal bone chondroblastoma represents a pathology that does not arise from, or have a growth pattern resembling other pathologies in the temporal bone.