We report a case of an intradural extramedullary malignant rhabdoid tu
mor (MRT) occurring in a 2-year-old girl. Histologically, immunohistoc
hemically and ultrastructurally, the tumor fulfilled the diagnostic cr
iteria for MRT. Many tumor cells contained typical cytoplasmic eosinop
hilic hyaline inclusions which were filamentous by electron microscopy
. Positive vimentin staining was observed immunohistochemically but ep
ithelial markers were negative. Extrarenal MRT are rare. Ten instances
in the central nervous system have been reported. This case is the fi
rst intradural MRT of the spinal canal. The differential diagnosis fro
m other tumors of this region is important because the prognosis and t
herapeutic approaches are greatly different.