Objective To evaluate the detection of alpha 5(Ⅳ) collagen chain of skin basement membrane in diagnosis of Alport syndrome among suspected patients. Methods Data of suspected patients with the detection of alpha 5(Ⅳ) collagen chain of skin basement membrane were retrospectively collected and analyzed from January 2007 to March 2008. Results A total of 254 suspected patients ranged from 1 to 71 years old with an average age of (25.85±17.03) years old were enrolled (male/female ratio, 0.76). There was no significant difference in average age between male and female. Abnormal alpha 5(Ⅳ) collagen chain expression of skin basement membrane was found by indirect immunofluorescense in 19 patients among whom 12 cases were negative and 7 cases were discontinous deposit. These 19 patients were diagnosed as Alport syndrome and the diagnostic rate was 7.5%. Conclusions The diagnostic rate of Alport syndrome by detection of alpha 5(Ⅳ) collagen chain in skin basement membrane is significant and helpful for early and differential diagnosis of Alport syndrome.