The incidence of pan-urothelial cell carcinoma (panUCC), which refers to the presence of both bilateral (UTUC) and bladder tumor (BT), is relatively low. However, the profile of a panUCC cohort of patients remains to be elucidated. We reviewed the data of consecutive UTUC patients who received treatment at our center from 1999 to 2012. Overall, 45 patients were included in this study, with a median age of 64.5 years. Fourteen patients initially presented with unilateral UTUC, 11 initially with BT, and the remainder with multiple tumors. Patients with UTUC were more likely to manifest higher rates of muscle invasion and larger-sized tumors. Five patients were treated with complete urinary tract exenteration (CUTE), and most patients (73.3%) received combined management with conservative and radical surgery. After a median follow-up of 77 months, 18 patients (40%) died including 15 (33.3%) due to cancer. Higher tumor stage was the only risk factor predictive of worse survival. Nineteen patients experienced local recurrence after conservative surgery. This study indicated that PanUCC involves either synchronous or metachronous presentation of tumors with a high risk of tumor recurrence, progression, and dissemination after conservative surgery.