To investigate the clinical characteristics and prognosis of patients with rare histological variants of bladder cancer (RHV-BC) in China. Patients diagnosed as bladder carcinoma with RHV in our center, from March 2009 and April 2019, were included. The univariate and multivariate COX regression model were used to evaluate the association between clinical characteristics and overall survival (OS). A total of 54 (1.4%) patients with RVH-BC were identified from 3803 potential patients with bladder cancer. The RHV classifications included micropapillary variant (MPV), sarcomatoid variant (SAV), neuroendocrine variant (NEV), nested variant (NV), plasmacytoid variant (PCV), and lymphoepithelioma-like variant (LEV), which were found in 19, 18, seven, six, three, and one patient, respectively. The mean of recurrence-free survival (RFS), cancer-specific survival (CSS), and OS of patients were 18.8 months, 37.0 months and 36.0 months, respectively. The multivariable analyses indicated that metastasis and T ≥2 were independent risk factors of OS. Besides, 84.4% (27/32) of patients who were receiving intravesical therapies (IVT) suffered recurrence. Patients with adjuvant chemotherapy (AC) had a recurrence rate of 64.7% (11/17). T≥2 and metastasis were independent risk factors of OS in patients with RHV-BC. Considering a high recurrence following transurethral resection of bladder tumor (TURBT) and IVT, early radical cystectomy (RC) might be performed for patients with RHV-BC. © 2020 Li et al.