Intracranial Extraskeletal Myxoid Chondrosarcoma in Fourth Ventricle

We present an extremely rare case of intracranial extraskeletal myxoid chondrosarcoma. A 36-year-old male presented with dizziness persisting for 2 weeks. MRI of the patient showed well-enhanced mass of fourth ventricle. The tumor was totally removed under telovelar approach. Pathology results confirmed an intracranial extraskeletal myxoid chondrosarcoma. Adjuvant radiotherapy was initiated one month after the surgery, and MRI followed 3 months after initial operation and showed no evidence of tumor recurrence.