Carcinosarcomas of the ureter are rare biphasic neoplasms, with distinct malignant epithelial and mesenchymal components. To the authors' knowledge there have been only 12 cases reported in the literature. Primary cartilaginous tumors of the ureter are also rare tumors. Thus far there has been a single case report of an extraskeletal mesenchymal chondrosarcoma of the ureter in the German literature. Herein a unique case is reported of ureteral carcinosarcoma in an 81-year-old woman presenting with gross hematuria in which the sarcomatous component had extraskeletal mesenchymal chondrosarcomatous differentiation. This is thought to be the first reported case of a carcinosarcoma with such a differentiation in the sarcomatous component.