A rare case of a primary Ewing's sarcoma arising from the intracranial portion of the right orbital roof is presented. Clinical presentation was that of a rapidly growing tender exostosis, associated with headaches. History indicated that the tumor may have been present for more than 1 year. Skull x-rays showed marked diastasis of the coronal suture and a "mottled" appearance on the right frontal bone suggestive of a sarcoma. Bone scan further confirmed the presence of a malignant bone tumor. Computerized tomography scan showed the intracranial extension of the tumor and a follow-up scan allowed us to see the response of the tumor to therapy. Only two other cases with details as to intracranial localization were found in the literature.