Non-urothelial bladder cancer patients represent a rare and challenging group. Advances in bladder cancer to date have largely been driven by studies investigating common urothelial bladder tumors. New evidence is emerging supporting lymphadenectomy in standard surgical management of muscle invasive bladder cancer. We aim to explore the utility of lymphadenectomy in non-urothelial bladder cancer.A systematic review of the available peer-reviewed literature on PubMed was performed using a PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) search strategy. Tumors included in our analysis were squamous cell carcinomas, adenocarcinomas, paragangliomas, melanomas and sarcomas.Our search strategy identified 8168 unique records and we included 135 full text articles in our final qualitative analysis. No comparative studies comparing lymphadenectomy outcomes in non-urothelial bladder tumors were identified. Practice of lymphadenectomy in combination with partial or radical cystectomy in the treatment of non-urothelial bladder cancer is relatively common. Pelvic recurrence following radical or partial cystectomy of non-urothelial tumors was more commonly reported in non-lymphadenectomy cohorts. The exception to this observation was the adenocarcinoma cohort.Current evidence supporting lymphadenectomy in the surgical management of bladder cancer is largely based on studies limited to urothelial cancer. Despite this, the practice of lymphadenectomy in non-urothelial cancer is common. We support lymphadenectomy in non-urothelial bladder cancer given the minimal risk associated with the procedure and the potential for improved survival.