Abstract: 　Objective　To improve the knowledge of primary gout in children. Methods Clinical data of a 12-year-old girl with primary gout was collected. Analysis of UMOD gene, REN gene and HNF-1β gene was performed using PCR and direct sequencing. Results The girl was admitted for 1-month history of left hallux pain accompanied with elevations of serum uric acid concentration and serum creatinine concentration. Several examinations showed serum uric acid/creatinine ratio was greater than 2.5. The fractional excretion of uric acid was 3.4%-6.6%. The X-ray showed that the proximal phalanxes of halluces were erosion. The diagnosis of renal biopsy was ischemic renal injury and chronic tubulointerstitial nephropathy. Blood uric acid concentrations of parents were normal, and the family history of gout was negative. Two single nucleotide polymorphisms (c.264C>T heterozygous and c.866-71 G>A heterozygous) in UMOD gene, 1 single nucleotide polymorphism (c.373+44C>G heterozygous) in REN gene, and 2 single nucleotide polymorphisms (c.100-50-49ins TCTG heterozygous and c.781-22T>C homozygous) in HNF-1β gene were detected. No pathological mutation was detected in these 3 genes. Conclusions This child is highly suspected to have primary gout caused by familial juvenile hyperuricemic nephropathy.