Abstract: Objective To explore the early diagnosis, treatment and prognosis of Dent disease. Method The clinical data of 6 children with Dent disease were retrospectively analyzed from January 2013 to December 2015. Results in 6 male chilren aged 3-10 years old, all of the children didn't have amino acid urine, urine glucose, or low potassium, while suffered with nephrocalcinosis. Two children had family history of proteinuria, one case was combined with microscopic hematuria, one showed glomerular focal segmental sclerosis (FSGS) in renal pathology, and one case accompanied with rickets and renal insufficiency. Two children were performed the gene detection and it showed the gene mutation site at c.731C>T (p.Ser244Leu) and c.848C>T(p.Pro283Leu) respectively. During the follow-up, the renal function was stable among all of the children. Conclusions The classical symptoms of Dent disease were low molecular weight proteinuria, hypercalcinuria, renal calcification, and so on. Gene detection is helpful in early diagnosis. Early diagnosis and treatment can improve the prognosis.