Objective To investigate the etiology,clinical presentation,diagnosis and treatment of inverted papilloma of the ureter. Methods The clinical data of 3 cases (all males,age range from 61 to 67 years) of inverted papilloma of the ureter were analysed retrospectively.All of 3 cases were diagnosed intraoperatively with the frozen section.Of them 1 case underwent segmental excision of the papilloma at ureter;1,nephrectomy plus subtotal ureterectomy;and 1,orthotopic excision of the papilloma. Results Postoperative pathology of the 3 cases confirmed the diagnosis of inverted papilloma of the ureter.No recurrence and metastasis were found postoperatively during a follow-up of 4.5,17 and 46 months in the 3 cases respectively. Conclusions Inverted papilloma of the ureter is a rare benign tumor with a good prognosis.Endoscopy and biopsy are the most important diagnostic procedures.Excising the local lesion and retaining the renal function is the preferred therapeutic choice.