An extraskeletal myxoid chondrosarcoma in the pineal region was studied by light and electron microscopy. The immunohistochemical positivity for S-100 protein, vimentin and collagen type II favored a chondrocytic origin of the tumor. In addition to the well-described ultrastructural features suggestive of a cartilagenous nature, this tumor had unusual lamellar inclusions in the rough endoplasmic reticulum. To the best of our knowledge, this is the first report of these special inclusion bodies in a myxoid chondrosarcoma.