A 20-year-old woman without neurofibromatosis presented with a 1-year history of headaches. At presentation, the patient was well oriented and did not show any focal neurological deficits. Ophthalmological examination revealed papilledema in both the optic fundi []. Visual acuity (VA) was 20/50 on the right side and 20/40 on the left, with an intraocular pressure of 17 mmHg on both sides []. Cranial computed tomography revealed a large tumor in the frontal lobe accompanied by focal erosion in the adjacent skull []. Magnetic resonance imaging (MRI) showed a tumor with broad-based attachment to the convexity dura mater. It appeared heterogeneous intensity on both T1- and T2-weighted sequences, accompanied by cyst components, measuring 82 × 65 × 70 mm in diameter, and inhomogeneously enhanced. No apparent dural tail was identified, and peritumoral brain edema was scant [-]. On the CISS sequence, the intraorbital ONs appeared prominent hyperintensity []. The widths and heights of the optic sheaths (OSs) and nerves were measured at the orbital apex as shown in and. The patient underwent microsurgical tumor resection. The tumor tissue was grayish, elastic soft and supplied by the peripheral branches of the middle meningeal artery at the site of the skull erosion. A gross total resection was achieved. Resected specimens revealed rhabdoid-shaped anaplastic cells with pleomorphisms. Focal microvascular proliferation and geographic necrosis were observed, in addition to four mitotic figures identified in 10 high-power fields []. Immunohistochemical examination showed positive staining for vimentin, S100 protein, progesterone receptor, and glial fibrillary acidic protein but negative for epithelial membrane antigen, CD34, STAT6, and Melan A. The MIB-1 index accounted for 50% [-]. These findings highly suggested RM. The patient’s postoperative course was uneventful. Her VA and IOP on postoperative day (POD) 6 were 20/17 and 10 mmHg, respectively []. On the CISS sequence performed on POD 5, prominent hyperintensity in the ONs showed remarkable resolution []. Furthermore, measurements of the OSs and nerves revealed a clear decrease in the width of the right ON. No alterations were observed in the OS measurements []. Although she did not receive immediate prophylactic radiotherapy, she remained under close surveillance, with MRI scans repeated every 6 months.