We report the first case of extra-axial anaplastic meningioma with direct orbital extension for differential diagnosis of orbital tumors.

An 83-year-old woman presented with a protruding left eye and a palpable mass on the forehead. A brain computed tomography scan revealed a huge sarcomatous mass that had directly invaded the surrounding tissues. We removed the tumor by craniotomy and found that it involved the extradural and intradural spaces, brain parenchyma, subcutaneous tissue, left temporalis, orbital roof, and the other frontal lobe across the midline. A cranioplasty was performed, and the orbital roof was reconstructed with artificial bone and bone cement. The final histological diagnosis was anaplastic meningioma. The left eyeball was restored to its normal position 1 month after the surgery. Also, visual acuity and eye movement of the left eye were preserved.

Direct orbital extension of originated extra-axial cerebral convexity anaplastic meningioma is rare but should be considered in the differential diagnosis of a rapidly growing orbital mass.