Abstract: Objective To explore the diagnosis and treatment of Dent’s disease. Methods The clinical characteristics, treatment process and disease-causing gene mutation were retrospectively analyzed in 6 pediatric patients with Dent’s disease misdiagnosed of nephritic syndrome from January 2014 to August 2015. Results In these 6 male patients aged 4.5-9.8 years old, the main clinical manifestations were nephropathy-level of proteinuria and transient low serum albumin (26-30 g/L) without obvious edema or high serum cholesterol. In 4 patients who had renal biopsy, 2 cases showed mesangial proliferative glomerulonephritis and other 2 cases showed focal segmental glomerulosclerosis. All of 6 patients were treated with at least one immunosuppressive agent after resistance to full dose of hormone and no changes in proteinuria were observed. After admission, the indexes of early renal damage and urinary protein electrophoresis pointed to low-molecular proteinuria. The ratio of alpha 1 micro albumin (α1-MG) / micro albumin (MA) (the early renal damage index) was > 1, there was hypercalciuria, and renal function was normal. The B ultrasonography showed renal calcification in 2 patients. The findings in all the patients were in accord with the clinical diagnosis of Dent’s disease. Further genetic analysis confirmed the presence of CLCN5 gene mutation in these 6 patients. Conclusion As a type of rare inherited renal tubular disorder, Dent’s disease is easily misdiagnosed, to which pediatricians need to pay attention. The early renal damage index, α1-MG/MA > 1, can be regarded as one of the diagnostic criteria of renal tubular proteinuria.