Renal transplantation in Alport syndrome is one of common hereditary disorder with progressive nephritis due to glomerular lesion. In Alport syndrome one or more normal GBM antigen is lacking. After kidney transplantation, anti-GBM antibody formation leads to anti-GBM nephritis and RPGN in allograft. In this study we followed up clinical courses of kidney transplantation in 11 patients with Alport syndrome and compared the results with 430 patients without Alport syndrome in GHAEM Hospital of MASHAD (IRAN). We don't have any cases of anti-GBM nephritis.2 patients with allograft dysfunction in kidney transplantation biopsy showed chronic rejection and 1 patient secondary to renal artery thrombosis lost his allograft. Graft survival and patient survival was comparable to the results in the patients with renal failure from other causes.