先天性肾病综合征是一种少见的常染色体隐性遗传性疾病.现报告如下.女,2月,以腹大2月入院.出生后7天会阴部肿胀渐延及全身,先后两次住某医院.按“新生儿硬肿症”治疗,效果不著并腹部逐渐增大.患儿系第3胎,足月顺产.出生体重3.5公斤.长姐4岁,健康.次姐生后1周出现浮肿、腹水,于5月龄时合并“肺炎”死亡.其父母健康,非近亲婚配.体检:发育 Congenital nephrotic syndrome is a rare autosomal recessive genetic disease are reported as follows. Female, February, admitted to the abdomen in Feb. 7 days after birth perineal swelling gradually extended and the whole body, has lived twice A hospital. According to “neonatal scleredema treatment”, the effect is not with the abdomen gradually increased .Small pediatric third-line, term full-term birth .Birth 3.5 kg. Sister 4 years old, healthy. Edema and ascites were seen in the week with death of “pneumonia” at 5 months of age, healthy parents and unmarried marriage.