A case is reported of unilateral proptosis of the left eyeball as a unique clinical presentation of primary angiosarcoma of the skull involving the sphenoid and frontal bones and roof of the orbit on the left side of a 32-year-old man. The intraosseous, extradural non-encapsulated, and friable tumour contained cytoplasmic factor VIII-related antigen. After complete removal of the tumour the patient has been well for 10 months of the follow-up period. This case brings the total number of reported primary angiosarcomas of the cranium to nine. All patients were male and most of them were young. The average age was 24 years. The frontal bone seems to be the site of predilection for cranial angiosarcomas. Surgical extirpation, radiation, or combined surgical removal and radiation have been used for treating angiosarcoma of the skull with variable results. It is suggested that angiosarcoma of the skull has a worse prognosis than angiosarcoma of bones other than the cranium because the patient may die of secondary neoplastic involvement of the brain.
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