Meningiomas arising from accessory nerve sheath without dural attachment are rare. To date, only 5 cases are described in the literature. A 53-year-old male presented with long history of occipital pain and headaches. Magnetic resonance imaging revealed a small intradural extramedullary contrast enhanced tumor at the level of foramen magnum. The patient underwent microsurgical resection through minimally invasive midline suboccipital approach. According to intraoperative findings, cystic tumor arose from the left accessory nerve without dural attachment. Gross total resection was achieved without damage to the nerve. Histological analysis revealed angiomatous meningioma. Postoperative period was uneventful without new neurological symptoms. Meningiomas can rarely arise from accessory nerve sheath and mimic schwannoma. These tumors may be totally resected without damage to accessory nerve using minimally invasive surgical approaches.