Behçet's syndrome (BS) is a rare systemic vasculitis that involves multiple systems and organs.Owing to the long-term prescription of immunosuppressive drugs, patients with BS are prone to urothelial tumors. However, surgical treatment in these cases could be dangerous because of the potential BS-related vasculitis, bleeding, and anastomotic necrosis. Literature reported that only five radical cystectomy cases had been performed on patients with BS, whereas the perioperative mortality was 40%. Moreover, perioperative managements on these cases were less discussed due to their multidisciplinary nature and rarity. We herein report a 54-year-old patient with a 13-year history of BS, diagnosed with sarcomatoid urothelial carcinoma and successfully underwent laparoscopic radical cystectomy with bilateral ureterocutaneostomy. No evidence of tumor residual or ureterocutaneostomic necrosis was reported in the subsequent three months of followup. In this case, the advantages of minimally invasive surgery in dealing with the parailiac lymph nodes were demonstrated. This article presented our thoughts, strategies, and experience on ensuring the patient's perioperative safety, as well as the selection of urinary diversion. We reviewed the literature in both English and Mandarin Chinese. Currently, two published articles describe the adoption of cystectomy in treating bladder cancer patients complicated with BS, whereas not any had shared the experience of using minimally invasive surgery. Hopefully, it could offer updated and enlightening significance to all related medical practitioners.