小儿先天性泌尿道器官畸形并非少见,它的预后与诊断的早晚密切相关,如能在严重合并症发生之前确诊,经外科矫治或相应的治疗常可取得较满意的效果。本文报告了本院收住的20例病例,其中单侧肾缺如2例,先天性肾囊肿1例,肾及输尿管畸形2例,重复肾、输尿管异位开口2例,先天性输尿管狭窄和扭转肾3例,胱膀憩室1例,脐尿管未闭1例,后尿道瓣膜1例及其它尿路畸形7例。小儿先天性泌尿系畸形的临床表现多种多样,有的患者可终身无症状,只有在手术或尸检时才被发现,有的可合并肾积水、感染,最终发生尿毒症而死亡。在本文20例中有表现尿路感染、血尿、腹部肿块、尿淋漓、尿失禁、排尿困难、生长迟滞、浮肿、输尿管和尿道异位开口,腹痛和高血压等症状的。诊断主要依据靠静脉肾盂造影(IVP),超声 Congenital urinary tract organ malformations in children is not uncommon, and its prognosis is closely related to the diagnosis of the morning and evening, as can be diagnosed before the occurrence of serious complications, the surgical treatment or the corresponding treatment can often get more satisfactory results. This article reports 20 cases admitted to our hospital, including 2 cases of unilateral renal failure, 1 case of congenital renal cysts, 2 cases of renal and ureteral malformations, duplication of kidney, 2 cases of ureteral ectopic opening, congenital ureteral stricture and 3 cases of torsion kidney, 1 case of cyst diverticulum, 1 case of uvularis patent, 1 case of posterior urethral valve and 7 cases of other urinary malformations. Children with congenital urinary tract malformations varied clinical manifestations, and some patients can be life-long asymptomatic, only in surgery or autopsy was found, and some may merge hydronephrosis, infection, and eventually uremia and death. In this paper, 20 cases showed urinary tract infection, hematuria, abdominal mass, urine dripping, incontinence, dysuria, growth retardation, edema, ureter and urethral openings, abdominal pain and hypertension and other symptoms. The diagnosis is mainly based on intravenous pyelography (IVP), ultrasound