Abstract: Mutations in the genes encoding the α,β, and γ subunits of the epithelial sodium channel in the nephron are the cause of Liddle syndrome. While existing literature extensively describes cases resulting from gene mutations in the β and γ subunits, reports on Liddle syndrome associated with mutations in the α subunit gene are relatively scarce. This article primarily reports a case of Liddle syndrome caused by a mutation in the α subunit gene, describing its clinical characteristics, treatment, and prognosis, aiming to provide practical insights for the clinical management of this condition.