Abstract: Gitelman syndrome (GS) is an autosomal recessive disease of salinized renal tubules, mainly characterized by hypokalemic alkalosis, hypopagnesemia, hypocalcinuria, and secondary aldosteronism. In this study, we reported the diagnosis and treatment of a child with GS misdiagnosed as diabetes insipidus. Meanwhile, the clinical manifestations, diagnostic methods, treatment and follow-up strategies of GS were discussed through literature review, and the importance of genetic testing in the diagnosis of this disease was emphasized. Clinicians should improve their understanding of GS in their work, and make early diagnosis and treatment of GS.