A thirteen-year-old boy with Ewing's sarcoma was presented. The treatment was considerably delayed because of the initial diagnostic difficulties due to its insidious presentation and the unwillingness of the child's parents to further investigations and treatment. During a three-year delay, the tumor turned out to be a gross painful mass from a painless lesion. Cranial computed tomography and magnetic resonance imaging with contrast showed a destructive lesion extensively involving the right mandible and the skull base, with invasion to the intracranial space. Facial and intratemporal portions of the mass were removed totally, but the intracranial extension could not be totally excised because of cavernous sinus involvement. Histologic and immunohistochemical findings were consistent with the diagnosis of Ewing's sarcoma. The patient received systemic chemotherapy and radiotherapy following surgery. A month after radiation therapy, radiologic investigations showed a lung mass suggestive of metastasis. He died two days after hospitalization, from an intracranial hemorrhage associated with the intracranial mass.

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