Alport Syndrome (AS) is an inherited kidney disease with progressive kidney failure. We investigate the potential of hyperpolarized 13C MRI to be used as an non-invasive technique for detecting early abnormal changes in renal metabolism. Hyperpolarized [1-13C]pyruvate imaging was applied to AS and wild-type mice at 4- and 7-week-old. Normalized Lactate in AS model was significantly lower at 7-week compared to 4-week. This abnormal renal metabolism in AS mice was consistent with PEPCK analysis, which showed increased expression level as the disease progressed. We demonstrated the feasibility of this technique for assessing early renal function in mouse model of AS.