Intramedullary teratoma is a rare neoplasm. To our knowledge only 10 cases have been reported so far, eight of mature teratoma and two of immature. We present a new case of mature teratoma located in the conus medullaris. After 2 years increasing complaints and worsening neurologic deficits, the neoplasm was discovered by magnetic resonance imaging and was completely resected surgically. Similar cases are reviewed and the clinical, radiological, surgical and morphological aspects of this lesion will be discussed. In the majority of cases, the tumour has an indolent growth rate and recovery after resection is good.