The association between Klippel-Trenauney syndrome (KTS) and bladder hemangiomas is rare. The most common clinical manifestation is hematuria. The diagnosis is made from the characteristic cystoscopic appearance of the tumor. We report the case of a patient presenting recurrent macroscopic hematuria in the context of KTS. A cystoscopic evaluation revealed bladder hemangiomas. A conservative approach consisting of bladder irrigation and close follow-up was chosen as therapy. Conservative treatment of bladder irrigation and close follow-up is the recommended initial treatment of moderate and infrequent episodes of hematuria in this context. The more invasive therapeutic options have to be considered especially for frequent or life-threatening episodes of hematuria. This case suggests that conservative treatment may be effective in treating moderate and infrequent episodes of hematuria due to bladder hemangioma in the context of KTS. Further studies are required to adequately establish the effectiveness, limitations, and complications of each approach.