先天性巨输尿管症又称原发性巨输尿管症或输尿管末端功能性梗阻。本文报告5例,均经膀胱镜、静脉排泄造影和膀胱造影而确诊。2.例手术切除功能性梗阻段,扩张段剪裁缝合后与膀胱再植。2例保守观察,1例行输尿管扩张治疗,经4个月至67个月随访观察,效果良好。我们认为本病治疗可首选扩张疗法,严密观察经过。如扩张治疗失败,再考虑手术治疗。 Congenital giant ureter, also known as primary giant ureter or ureter end of functional obstruction. This article reports 5 cases, were confirmed by cystoscopy, venous drainage and cystography. 2. Cases of surgical resection of functional obstruction segment, expansion segment tailored suture and bladder replantation. 2 cases of conservative observation, 1 case of ureteral dilatation treatment, after 4 months to 67 months follow-up observation, the effect is good. We believe that the treatment of this disease may be the preferred dilation therapy, closely observed after. Such as the expansion of treatment failure, and then consider surgery.