Abstract: Alport syndrome (AS) is an inherited type Ⅳ collagenrelated disorder that can involve the kidneys, cochlea, eyes and eventually lead to impaired organ function and endstage renal disease. There is still a lack of etiologic therapy. As a symptomatic therapy, angiotensin converting enzyme inhibitors (ACEI) & angiotensin Ⅱ receptor blockers (ARB) have shown encouraging therapeutic effects in reducing proteinuria and delaying the progression of renal failure, patients would benefit more from early clinical intervention. We reviewed the research progress of ACEI／ARB therapy in animal models and clinical trials, aiming to promote the standardized clinical use of ACEI／ARB to benefit more AS patients.