A case of CNS gliomyosarcoma, in a 71-year-old female with skeletal muscle differentiation is presented. The tumor was composed by two cell types: one showed features typical of glial cells, the other was constituted by elements having immunohistochemical positivity with desmin, sarcomeric actin, myoglobin and myogenin antisera. It is postulated an origin from a cell capable of dual differentiation.

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