and Importance: Malignant craniopharyngioma (MCP), first described in 1987 by Akachi, is an exceedingly rare disease that is characterized by cytological atypia, high mitotic activity and poor prognosis. By now, only 19 cases of MCP were reported of which only 3 cases were primary type. Clinical Presentation: We presented the diagnosis and treatment of fourth primary MCP. The patient was preoperatively diagnosed as cra-niopharyngioma (CP) and underwent a total tumor resection. Histopathological

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... examination showed that tumor cells had an increased nuclear-cytoplasm ratio, a high mitotic activity and Ki-67 > 20%. The patient was diagnosed as MCP. Adjuvant radiotherapy was followed after surgery and the patient remained no progression in the next 8 months follow-up. Conclusion: The mechanism, diagnosis and treatment for MCP is still challenging. Surgical resec-tion followed by radiotherapy has demonstrated an effective treatment and chemotherapy still needs researches to demonstrate its therapeutic efficiency.