Document Type : Case Reports


Dept. of Internal Medicine, Sary University of medical sciences, Sary, Iran


  Anderson-Fabry disease is a rare inherited X-linked lysosomal storage disease caused by deficiency of the enzyme alpha-galactosidase A. Hereby we report a 39 year old male that presented with proteinuria and edema. Histopathologic, immunofluorescence and ultrastractural examination of renal tissue were in favor  of  Fabry disease in associate with IgA nephropathy. Fabry's disease associated with IgA nephropathy apparently is extremely rare, and the present case is among few reported cases in literature.