ObjectiveTo discuss the treatment of a rare case of Klippel-Trenaunay syndrome associated with bladder hemangioma and conduct a brief literature review. MethodsA 23-year-old female manifested episodes of painless hematuria for 1 month.A biopsy by cystoscopy diagnosed angiomatosis.The patient had a history of left lower limb hemangioma for 23 years,and underwent 3 surgical operations at 3 months old,3 years old,and 13 years old.The left leg was longer than the right leg.There was some soft tissue hypertrophy in the left lower extremity.Ultrasonography showed a bladder mass.Cystoscopy revealed a 3 cm x 5 cm sized hemangiomatous on the left lateral and anterior wall of the bladder. ResultsThe patient was diagnosed with bladder hemangioma and Klippel-Trenaunay syndrome.Partial cystectomy was performed.The pathological diagnosis was bladder intramuscular hemangioma. ConclusionKlippel-Trenaunay syndrome associated with bladder hemangioma can be treated by partial cystectomy.