? Hereditary renal tubular alkalosis in children is a rare renal tubular disease. It is a clinical syndrome caused by protein dysfunction resulted from single gene mutation of ion channel in renal tubular epithelial cells. Bartter syndrome, Gitelman's syndrome and Liddle's syndrome are among the more common types, all of which are hereditary potassium loss diseases clinically characterized by renal tubular alkalosis. In this paper, the pathogenesis, clinical manifestations and the latest progress in diagnosis and treatment were reviewed.