【摘要】 目的 探讨婴幼儿常见泌尿系统先天发育异常的超声特征。 方法 回顾性分析2008年9月17日-2010年6月1日体检的婴幼儿中所发现的各种泌尿系统先天性发育异常的超声表现。 结果 发现泌尿系统先天性发育异常88例，其中肾缺如50例，多房性肾囊性变10例，多囊肾2例，肾旋转不良1例，重复肾10例，输尿管囊肿6例，异位肾7例，融合肾2例。 结论 婴幼儿常见的泌尿系统先天性发育异常有较特异的超声表现，超声检查是筛查婴幼儿泌尿系统先天性发育异常的首选影像学检查方法。【Abstract】 Objective To study the ultrasonic characteristics of congenital malformation of infantile urinary system. Methods We analyzed various ultrasonic appearances of congenital malformation of infantile urinary system in our hospital in the recent two years retrospectively. Result We found 88 cases of congenital urinary system malformation in all infants, including 50 cases of renal agenesis, 10 cases of multi-cystic kidney, 2 cases of policystic kidney, 1 case of malrotation of kidney, 10 cases of duplex kidney, 6 cases of ureter cyst, 7 cases of ectopic kidney, and 2 cases of fused kidney. Conclusion Special ultrasonic appearances can be detected for congenital malformation of infantile urinary system, so ultrasound can be regarded as the first-line iconographical examination for the disease.