In a 57-year-old female patient, a solitary skull metastasis of endometrial stromal sarcoma of the uterus was found under the left parietal region of the cranium 1 year after total removal of the primary lesion. The histological features of the skull mass were similar to those of the original tumor. Fifteen months after excision of the skull metastasis the patient remains asymptomatic. This is the first such case reported.