Abstract: 　Objective　To explore the clinical characteristics and gene mutation of congenital nephrotic syndrome of the Finnish type (CNF). Method　The clinical data and gene test results of one CNF child and her siblings and parents were retrospectively analyzed and relevant literature were reviewed. Results　A female neonate who was born at gestational age of 31 weeks with birth weight of 1560 g presented with hypoproteinemia, proteinuria, and progressive pitting edema 3 days after birth. Two heterozygous mutations in NPHS1 gene, c.3478C > T (exon 27) and c.2515delC (exon 19), were found in the neonate by Gene detection. The results of routine urinalysis of her father, mother and twin sister were normal. The mutation of c.3478C＞ T was inherited from her father and c.2515delC (exon 19) from her mother, and her twin sister had no mutation. The two genetic mutations have been reported as pathogenic gene mutations, while c. 2515delC has not been reported domestically. Conclusion The gene mutation spectrum of CNF in China has been expanded by the discovered mutation gene.