Aim: To perform a systematic review of the literature regarding epidemiology, diagnosis, management and prognosis of bladder cancer in the neuro-urological patient population, in order to serve as a basis for future recommendations and research. Methods: A systematic review was performed according to the PRISMA-Preferred Reporting Items for Systematic Reviews and Meta-Analyzes Statement. Embase was searched for studies providing data on epidemiology, diagnosis, management and prognosis of bladder cancer in neuro-urological patients. Results: After screening 637 abstracts, 15 studies (13 retrospective and 2 prospective studies) were included in this study. We identified 332 patients (0.3%) who were diagnosed with bladder cancer. This mostly affected mostly men (59.3%) and spinal cord injured patients (98.8%). Mean age at diagnosis was 56.1 years. Bladder cancer occurred after a long period of evolution of the neurological disease (24.9 years). Gross hematuria was the predominating presenting symptom (31.6% of cases). Indwelling urethral or supra-pubic catheters were used in 44.5% of patients. The most frequent histological subtype of bladder cancer was transitional cell carcinoma (53.1%), followed by squamous cell carcinoma (33.5%). Muscle-invasive bladder cancer was reported in 67.7% of patients. The mean cancer-specific mortality rate was of 47.1%. Conclusions: The prevalence and high mortality rate of bladder cancer in neurourological patients underlines the importance of long-term follow-up in this specific population. This highlights the necessity of further studies in this field.