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A 12-year-old boy presented with primary Ewing's sarcoma of the occipital bone manifesting as intermittent high fever and local pain in the occipital region. Plain skull x-ray films disclosed an unclear lytic lesion in the occipital bone. Computed tomography and magnetic resonance imaging demonstrated the irregularly enhanced mass. The tumor was removed totally. He received intensive chemotherapy and radiation therapy postoperatively. No recurrence or metastasis has been noted, and he was in good condition 18 months after the operation.
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