OBJECTIVES/HYPOTHESIS Chondrosarcomas of the jugular foramen are extremely rare tumors. Our review of the literature revealed eleven previously reported cases. The aim of this study is to describe the presenting symptoms, radiographic findings, operative procedures, and postoperative outcome of five histologically confirmed cases of chondrosarcomas arising from the jugular foramen. A review of the literature is also presented. STUDY DESIGN Retrospective study of an quaternary referral otology and skull base private center. METHODS Five cases of surgically treated and pathologically confirmed jugular foramen chondrosarcomas were identified. The follow-up of the series ranged from 23 to 42 months (mean, 32.8 +/- 7.7 months). RESULTS A single stage procedure was adopted in all the cases. Two patients underwent type A infratemporal approaches, one patient underwent a transotic approach extending to the neck with ligature of the internal jugular vein, one patient underwent a petro-occipital transigmoid approach, and one patient underwent a combined petro-occipital transigmoid-transotic approach. Gross total tumor removal was achieved in all patients. The most common complications were lower cranial nerve deficits. To date, no recurrence or residual tumors have been observed at radiological controls. CONCLUSIONS We believe that the primary treatment for chondrosarcomas of the jugular foramen is gross total surgical resection of the tumor. It is our philosophy to reserve postoperative radiotherapy for patients with histologically aggressive tumors, as well as in cases with subtotal resection and recurrent tumors.