Sarcomatoid urothelial carcinoma (SaUC) of the ureter and kidney is a rare variant of urothelial carcinoma (UC) as it is both epithelial and mesenchymal. Due to rapid progression and widespread metastasis, the prognosis of SaUC is extremely poor. We report a rare case of SaUC involving the left ureter and kidney of a 51-year-old woman. Microscopic hematuria and anemia were noted for the past 7 years. The patient reported a history of taking Chinese herbal medicine since the age of 2. Initially, she visited our gastrointestinal outpatient department due to fever and upper abdominal pain radiating to her back for more than 2 weeks. On abdominal ultrasound she was found to have a tumor on her left kidney. She was referred to our genitourinary outpatient department. Computed tomography imaging of the abdomen revealed a large tumor on her left kidney with scattered calcifications. Subsequently, left nephroureterectomy, adrenalectomy, and bladder cuffectomy were performed. A pathologic diagnosis of SaUC of the left ureter and kidney was established. Unfortunately, the disease progressed rapidly, and due to intraperitoneal carcinomatosis and diffuse lung metastasis, the patient died on the 32nd day after surgery. Risk factors such as genetic susceptibility, environmental carcinogens of aristolochic acid, diagnosis process and treatment recommendations were discussed. This case highlights that kidney tumors may occur when microscopic hematuria and chronic anemia are discovered. A further evaluation with abdominal US and CT are required.