This article reviews the pathology, clinical course and management of cavernous angiomas in the brain stem and spinal cord. Both lesions have been diagnosed with increasing frequency as a result of magnetic resonance image scanning. Brain stem lesions tend to present dramatically; their treatment remains microsurgical excision despite some studies that have looked at the use of radiosurgery. Spinal lesions are either extra-, or more commonly, intramedullary. Intramedullary cavernomas present with a wide spectrum of symptoms ranging from acute haematomyelia to presentations that mimic demylelinating conditions; extramedullary cavernous angiomas tend to produce radicular symptoms or subarachnoid haemorrhage. Both are treated by microsurgical excision.