目的通过分析1例先天性右侧巨输尿管并右肾缺如病例,提高对先天性巨输尿管并肾缺如疾病的认识。方法对新疆和田地区洛浦县人民医院收治的先天性巨输尿管并右肾缺如病例资料进行分析,复习相关文献资料,总结先天性巨输尿管的诊断经验。结果吸取对先天性巨输尿管误诊教训,提高了诊断水平。结论诊断疾病不要过分依赖辅助检查,要拓宽思路,考虑全面。 OBJECTIVE To improve the understanding of congenital giant ureter and renal insufficiency by analyzing one case of congenital right giant ureter and missing right kidney. Methods The data of patients with congenital giant ureter and right kidney who were admitted to Luopu County People’s Hospital in Hetian area of ​​Xinjiang Autonomous Region were analyzed. Relevant literatures were reviewed and the diagnosis experience of congenital giant ureter was summarized. The results of lessons learned on the misdiagnosis of giant ureter to improve the diagnostic level. Conclusion Diagnosis of diseases do not rely too much on ancillary examination, to broaden their thinking, consider the comprehensive.