Cavernous malformations are being increasingly well recognized throughout the central nervous system, where they compose approximately 1% of all vascular lesions and 15% of all vascular malformations. These malformations are uncommon in the spinal column and rarely found within the spinal cord. There have been only a few isolated reports of surgical resection of intramedullary spinal cord cavernous malformations. A series of six patients with intramedullary spinal cord cavernous malformations is described; all were treated by complete surgical excision, and all had a good or excellent outcome with partial or complete relief of neurological symptoms. There were no surgical complications, although transient postoperative neurological worsening was common. The clinical, radiological, and surgical features of these patients are discussed. It is concluded that intramedullary spinal cord cavernous malformations are an uncommon cause of progressive myelopathy that can be safely and effectively treated by surgical excision.