Atypical choroid plexus papillomas are rare in adults, especially in an extraventricular location.We present a case of a 42-year-old male patient suffering from a foramen magnum choroid plexus papilloma, which resembled meningioma in an initial evaluation, who developed dizziness, hearing loss, and gait disturbance. The patient was treated with suboccipital craniotomy and the tumor was partially removal. We describe the technique and postoperative complications. We also review other published cases of atypical choroid plexus papillomas of the posterior fossa in adults.