OBJECTIVE:Angiosarcoma is a rare neoplasm that uncommonly involves the cranium. We report the second case in the literature of a primary right spheno-orbital malignant angiosarcoma.CLINICAL PRESENTATION:This 43-year-old man experienced a 3-month history of rapid growing temporal mass. The results of his neurological examination were normal. Neuroimaging revealed an intensively enhanced right spheno-orbital lesion, with destruction of the greater wing of the sphenoid bone and extension into the orbit, the infratemporal fossa, and the temporal fossa with infiltration of the dura mater but without parenchymal abnormality.INTERVENTION:The tumor was first biopsied and then totally removed, with exenteration of the right eye. The histological features were typical of angiosarcoma with immunohistochemical evidence of Factor VIII-related antigen produced by tumor cells. Neither radiotherapy nor chemotherapy was performed. The patient remained well during 16 months of follow-up, without evidence of recurrence on magnetic resonance images.CONCLUSION:We emphasize the benefit of wide surgical resection without systematic complementary treatment (radiotherapy and/or chemotherapy) in a case of primary angiosarcoma of the cranium. The literature is reviewed.