目的 :探讨先天性肾发育不全合并其它尿路畸形的发病特点 ,阐述定位诊断的方法和治疗经验。方法 :回顾 4 2例肾发育不全的临床资料 ,对该病的X线征象、合并畸形和手术方法进行分析。结果 :3 9例施行手术 ,其中 3 4例作患肾切除术 ,5例作输尿管移植术 ,疗效满意。结论 :静脉尿路造影 (IVU)、B超、逆行造影是诊断本病的主要方法。对患肾功能差 ,对侧肾功能正常者 ,主张作肾切除术。 Objective: To explore the incidence of congenital renal dysplasia combined with other urinary tract malformations, elaborating the method of positioning diagnosis and treatment experience. Methods: The clinical data of 42 cases of renal dysplasia were retrospectively analyzed, and the X - ray signs, combined deformity and surgical methods were analyzed. Results: 39 cases underwent surgery, of which 34 cases were treated with nephrectomy and 5 cases were treated by ureter transplantation. The curative effect was satisfactory. Conclusion: Intravenous urography (IVU), B-ultrasound and retrograde angiography are the main methods of diagnosis of this disease. Right renal dysfunction, contralateral renal function were normal, advocated for nephrectomy.