Mixed inverted urothelial papilloma (IUP) and exophytic urothelial carcinoma (EUC) of the urinary bladder are a rare feature consisting of benign IUP with areas typical of exophytic carcinoma. Reports guiding the pathological diagnosis and treatment of these urothelial neoplasms are limited. Here, we examined three cases of mixed IUP and EUC to better establish the clinicopathological features of this disease. We reviewed 3206 transurethral resection of bladder tumor cases from the Sun Yat-sen Cancer Center between January 2001 and December 2014. We identified three cases of typical mixed IUP and EUC (3/3206), giving an incidence of 0.09% at the single center. All of the three patients, a 41-year-old male, a 69-year-old male and a 74-year-old male, presented with hematuria. In addition to typical IUP of the urinary bladder, 3 cases showed areas of exophytic neoplasms. One of the cases with exophytic neoplasms was high-grade papillary urothelial carcinoma of the urinary bladder, while the other two were low-grade papillary urothelial carcinoma. All the three patients showed symptomatic relapse at follow-up. Our study reports for the first time the incidence at a single center of mixed IUP and EUC, and identifies morphological features to help guide pathological diagnosis and clinical treatment.