Primary adenocarcinoma of the upper tract urothelial cell carcinomas (UTUCs) is rarely reported. We report a case of primary adenocarcinoma of the ureter with elevated carbohydrate antigen 19-9 (CA-19-9). A 44-year-old male was admitted to the hospital for severe right flank and hypogastrium pain for 7 days without visible or microscopic haematuria. The patient underwent radical nephroureterectomy followed by chemotherapy with gemcitabine/ carboplatin and inorelbine/Adriamycin (ADM)/5-fluoro-2,4 (1 h, 3 h) pyrimidinedione (5-FU) and radiotherapy, and the level of CA19-9 decreased to normal. Histopathology revealed adenocarcinoma. The patient died of tumor pelvic metastasis after 6 months. A review of the literature is also reported.