A retrospective review of 552 patients with primary bladder cancer yielded 7 cases (1.2%) of pure squamous cell carcinoma. Their clinical presentations did not differ from conventional transitional cell carcinomas, gross hematuria being the most frequent complaint (6 cases). All the cases presented as flat tumors. They were large and frequently affected more than one topographic area. The trigone was almost always affected (6/7). Three cases were considered low and 4, high grade. There were no dysplastic changes on the peripheral mucosa in any case. At the time of diagnosis, all of them were deeply invasive (pT3 and pT4). Two cases were Stage III and 5 Stage IV, with involvement of lymph nodes and metastases to the lung (2 cases), liver (1 case), and bone (1 case). All the patients died of tumor and the overall survival was very poor (range 6-37 months; mean 17.4).