目的探讨新生儿尸检肾囊肿病的分型及临床病理特征。方法复习内蒙古妇幼保健院1987—2011年进行尸检发现肾囊肿病的新生儿或引产胎儿病例临床病理资料,并对其组织病理学及合并症进行分析。结果 11例肾囊肿病分别属Ⅰ、Ⅱ、Ⅲ型,并同时合并心脏、肺脏、消化道、外生殖器和骨发育异常。其中2例进行染色体分析后均为(46,XX),无异常。结论肾囊肿病为常染色体隐性遗传性疾病,常合并身体其他部位发育异常,严重威胁患儿健康,已生育过本病患儿的夫妇再次妊娠时应严密监测。 Objective To investigate the classification and clinicopathologic features of neonatal autopsy renal cysts. Methods To review the clinical and pathological data of neonatal or induced fetus fetuses with kidney cysts found in autopsy in MCH from 1987 to 2011 in Inner Mongolia MCH and its histopathology and comorbidity were analyzed. Results 11 cases of renal cysts were type Ⅰ, Ⅱ, Ⅲ, and combined with heart, lung, digestive tract, external genitalia and bone dysplasia. Two of them were (46, XX) after chromosomal analysis, no abnormalities. Conclusions Renal cyst disease is an autosomal recessive disease, often associated with dysplasia in other parts of the body. It is a serious threat to the health of children. Couples who have given birth should be closely monitored again during pregnancy.