Objective: Intracranial extraskeletal myxoid chondrosarcoma is a very rare tumor; up to date, only ten cases have been reported.

Case report: We present a case report of a 65-year-old female admitted to Zagazig university hospitals by right side hemiplegia of gradual onset and progressive course over three weeks. Magnetic resonance imaging (MRI) revealed left intra-axial parieto-occipital space occupying lesion, multiloculated, hypodense with central necrosis, heterogeneous enhancement, minimal brain edema, and causing a mass effect. The patient underwent subtotal resection using sonar guided craniotomy. The histopathological and immunohistochemical evaluation confirmed a diagnosis of extraskeletal myxoid chondrosarcoma. One month after initial discharge, the patient underwent radiotherapy. About 6 months after surgery, the patient was deteriorated and died.
Conclusion: Intracranial extraskeletal myxoid chondrosarcoma is a rare malignant cartilaginous tumor. Pathological diagnosis is the gold standard and radical excision is the standard treatment.