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A 65-year-old female presented with a rare intestinal leiomyosarcoma metastasis to the skull manifesting as a mass beneath the scalp. She was free of neurological and physical symptoms on admission. The tumor was totally removed with normal surrounding bone and dura. The histological diagnosis was leiomyosarcoma. Ultrastructural and immunohistochemical studies demonstrated the smooth muscle origin of the tumor. Such patients in good physical condition should be immediately treated surgically to achieve the best chance of survival.