No prior reports have focused on the natural history and long-term outcomes of intramedullary spinal cord cavernous malformations (ISCCMs) in children. To investigate the clinical characteristics and long-term outcomes of pediatric ISCCMs and identify the risk of hemorrhage. We retrospectively reviewed a series of 20 pediatric patients (<18 yr old) from a consecutive series of 254 patients with ISCCMs evaluated at a single institution. Of the 20 pediatric patients, 9 (45.0%) presented with a severe neurological and disability status. The annual hemorrhagic rate in pediatric patients was 8.2%/patient/year. After initial overt hemorrhage events, the annual overt rehemorrhage rate increased to 30.7%/patient/year. In 234 adult patients, the respective rates were 2.8% and 7.4%. Thoracic or lumbar level lesions (P = .002, OR = 3.425, 95% CI = 1.588-7.387) and rehemorrhagic events (P = .005, OR = 3.209, 95% CI = 1.415-7.279) were more likely to follow an aggressive course. There were no significant differences in the sex distribution, location and size of lesions, types of symptoms, likelihood of a severe neurological and disability status, or immediate and long-term postoperative outcomes between pediatric and adult patients with ISCCMs. The annual overt hemorrhage rate and rehemorrhage rate of ISCCMs were higher in affected children than in affected adults. Surgical resection of pediatric ISCCMs remains the preferred therapeutic option and provides favorable outcomes. Copyright © 2019 by the Congress of Neurological Surgeons.