Myxopapillary ependymomas (MPEs) have historically been thought to be benign tumors occurring most frequently in adults. Only 8 to 20% of these tumors occur in the first two decades of life, making this tumor a rarity in pediatric neurosurgery. Five patients with intraspinal MPEs were treated by the authors between 1992 and 2003. Four (80%) of these five patients suffered from disseminated disease of the central nervous system (CNS) at the time of presentation; this incidence is much higher than that reported in the combined adult and pediatric literature. Combining five pediatric case series reported in the literature with the present series, the authors review a total of 26 cases of pediatric patients with intraspinal MPEs. In nine cases (35%) CNS metastases occurred. In those cases in which patients underwent screening for CNS tumor dissemination, however, the incidence of disseminated disease was 58% (seven of 12 patients). In pediatric patients MPEs may spread throughout the CNS via cerebrospinal fluid pathways; therefore, MR imaging of the entire CNS axis is recommended at both presentation and follow-up review to detect tumor dissemination.