A 35-year-old man presented with pain in the right shoulder and neck for 18 months. The neurological examination revealed complete accessory nerve palsy on the right side without further deficits. Magnetic resonance imaging showed a right parapharyngeal tumour expanding into the posterior fossa through the jugular foramen without dural attachment and absence of invasion into the middle ear cavity or internal auditory meatus. Intraoperative inspection disclosed a tumour originating from the accessory nerve. Histological diagnosis revealed a meningothelial meningioma with invasion of the epineural space. To the knowledge of the authors this is the first report of an accessory nerve meningioma in the jugular foramen associated with a posterior fossa component and extension into the parapharyngeal space.