Intramedullary spinal schwannomas are extremely rare. We describe a case of a 13-year-old female who was found to have an intramedullary schwannoma at craniovertebral junction along with incidental bilateral acoustic neuromas, which was excised completely. To our knowledge, this is the first report of a case of intramedullary schwannoma in association with NF-2 located at the craniovertebral junction. The literature has been reviewed with emphasis on radiological imaging, histopathological diagnosis and technique to achieve complete microsurgical excision.