We reviewed the clinical, radiographic, and pathologic findings of ureteral transitional cell carcinoma (TCC) in a blackfoot disease (BFD) endemic area in southern Taiwan to increase understanding of tumor behavior in this area, which has a high incidence of urothelium carcinoma. From August 1995 to February 2002, 28 histologically proven ureteral TCCs from this area were eligible for study. There was an unusual female predominance (male:female ratio, 1:1.333). The urographic filling defects in the 28 ureteral TCCs were classified into four categories. An ovoid filling defect was significantly associated with non-invasive tumors (p = 0.003) and a trend toward low tumor grades (p = 0.073). The risk of ureteral TCC in this BFD endemic area of southern Taiwan remained high between August 1995 and February 2002. Urographic surveillance provides a simple, clear, inexpensive method to study the extent, location, and morphology of the ureteral mass. Detailed assessment of the image could be useful for preoperative planning and predicting prognosis. Large-scale, randomized, prospective clinical trials are needed to validate our results.