Sarcomatoid urothelial carcinoma of the urinary tract is associated with poor prognosis. The majority of sarcomatoid urothelial carcinomas are found in the urinary bladder, while a small minority have been shown to arise from the ureter. In the present case, a 72-year-old male patient was diagnosed with sarcomatoid urothelial carcinoma with chondrosarcomatous differentiation of the left ureter, and subsequently underwent nephroureterectomy and retroperitoneal lymph node dissection. Two lymph nodes from the renal hilum and one paraaortic lymph node were also found to have metastatic involvement; however, the patient refused to receive chemotherapy or radiotherapy and succumbed to bone and omentum metastasis at 6 months after the initial diagnosis. Sarcomatoid urothelial carcinoma of the ureter is uncommon. Even rarer is the presence of malignant heterologous elements, such as chondrosarcoma. The present study reports a rare case of sarcomatoid urothelial carcinoma with chondrosarcomatous differentiation of the ureter, as well as a review of the literature, in order to demonstrate the aggressive nature of this particular malignancy.