Two children and one young adult with extremity sarcomas demonstrating an aggressive clinical behavior are described. Histologically, all three tumors displayed features compatible with a deep-seated epithelioid sarcoma or with a malignant rhabdoid tumor of soft tissue. Immunohistologically, both vimentin and epithelial antigens were demonstrated; however, no desmin was detectable. In all three cases, the DNA profile was diploid. In one case, a trisomy of chromosome 2 was found in the tumor cells, a phenomenon also observed in embryonal rhabdomyosarcomas. It is concluded that, although the immunohistologic findings support the epithelioid character of the tumors, this chromosomal finding suggests a relationship with rhabdomyosarcomas and justifies the designation of rhabdoid.