A primary intraosseous cavernous hemangioma located at the sphenoid bone with extensive involvement of the orbital roof and the lateral wall of the orbit is very rare. A 48-year-old woman presented with progressive right exophthalmos and diplopia. CT showed a bony mass lesion in the right sphenoid bone extending to the orbital bone. MRI showed an abnormal lesion in the sphenoid bone, which was heterogeneously enhanced with gadolinium. All of the abnormal bone was surgically removed, and histological examination confirmed a cavernous angioma. We also present a brief clinical and radiological review of seven previously reported cases.