We describe an unusual soft tissue tumor occurring within the rectus femoris muscle of a 64-year-old woman. The site, size, macroscopic, and histological appearances were all consistent with an extraskeletal myxoid chondrosarcoma. However, the present case differs significantly from previous reports of this uncommon tumor in that electron microscopy did not show any evidence of chondroblastic differentiation. Furthermore, the cells failed to stain for vimentin while labeling intensely for neuron-specific enolase, contained large numbers of cytolysosomes having a multivesicular appearance, and focally produced an external lamina. Based on the typical histological appearances we conclude that this is an unusual variant of extraskeletal myxoid chondrosarcoma in which there is evidence of nerve sheath differentiation.