Abstract: Objective To investigate the clinical manifestations, diagnosis and treatment of Bartter syndrome in children. Methods Clinical data of 15 patients with Bartter syndrome in Children`s Hospital Affiliated to Chongqing Medical University was analyzed, and pertinent literatures were reviewed. Results Bartter syndrome is an autosomal recessive inherited renal disorder characterized by hypokalemia, hypochloremia, metabolic alkalosis, vomiting, growth retardation, the activation of the renin-aldosterone axis, normal blood pressure. Genetic analysis is the most reliable way for diagnosis. Comprehensive therapy with antisterone, indomethacin, catopril and potassium have remarkable effect. Conclusions Bartter syndrome should be considered when children have unreasonable continuous hypokalemia, hypochloremia, metabolic alkalosis and growth retardation. It can be clinically diagnosed by clinical manifestation and hydrochlorothiazide test, and genetic analysis is the most reliable way. It can be ameliorated by potassium and magnesium supplementation, antialdosterone medications, prostaglandin inhibitors and antisterone. Considering the following electrolyte disturbances, infections, growth retardation, kidney failure and even death，Bartter syndrome need lifelong treatment, early diagnosis and treatment is of the most importance.