Background: Primary Ewing's sarcoma (ES) arising from cranial bones is an extremely rare entity that accounts for only 1-4% of all ES cases.
Case description: A 21-year-old woman presented with ES of the skull affecting the occipital region. The patient underwent surgical excision following radiotherapy and chemotherapy. No recurrence or metastasis occurred over a 10-month follow-up.
Conclusion: ES reaches adolescents. The mainstay of treatment includes surgical removal of the tumor, followed by radio- and chemotherapy.
Keywords: Ewing sarcoma; Occipital bone; Skull.
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