小儿先天性巨输尿管合并肾缺如和膀眈外翻合并多发骨畸形者临床少见，现将笔者收治2例，报告如下。1病历摘要例1，女患，6岁。患儿自幼排尿困难，伴有腰痛。查体及实验室检查无异常发现。尿路造影见左侧肾脏无异常发现。输尿管充盈良好。右侧肾区无肾影，输尿管未显影，膀眈逆 Pediatric congenital giant ureter combined with kidney deficiency and bladder incision combined with multiple bone deformities are rare, now the author admitted to 2 cases, the report is as follows. 1 case summary 1, female, 6 years old. Children with dysuria since childhood, accompanied by low back pain. Physical examination and laboratory tests found no abnormalities. Urinary tract imaging showed no abnormalities in the left kidney. Ureteral filling well. The right kidney area without renal shadow, ureter is not developed, the contralateral bladder