Object. Intramedullary spinal cord cavernous malformations (CMs), once thought to be extremely rare, have been diagnosed more frequently since the advent of MR imaging. In the literature, however, only a few studies include more than 10 cases. The aim of this study was to discuss the clinical presentation of intramedullary spinal cord CMs and the outcome of microsurgery for these histologically benign but clinically progressive lesions. Methods. The authors retrospectively reviewed the records of 20 patients who underwent microsurgery for Ultramedullary spinal cord CMs. All patients had undergone pre- and postoperative MR imaging, and they were all treated using microsuraical resection. The diagnosis of spinal cord CMs was based on pathological criteria. The pre- and postoperative neurological states of the patients were classified according to the McCormick scale and Frankel scale. The microsurgical outcomes are presented and discussed. Results. In most cases, CMs can be diagnosed on the basis of MR imaging findings, since these lesions have certain characteristic imaging patterns. Patients with intramedullary spinal cord CMs may present with either a rapid, acute onset of symptoms or slow, progressive neurological decline. The CMs in 19 of 20 patients in this series were totally resected, and most patients neurologically improved postoperatively. As previously reported, the authors confirm that the treatment of choice for symptomatic intramedullary CMs is total removal of the lesion to avoid recurrence and the possibility of further hemorrhage. Conclusions. This study has defined the clinical features of symptomatic intramedullary spinal cord CMs. Surgery is the mainstay treatment. Surgical outcome is associated with low mortality with a high probability of functional recovery, especially when symptoms are not severe and are of relatively recent onset. (DOI: 10.3171/2011.11.SPINE11536)