We described here one sibling with focal glomerular sclerosis. Proteinuria was noticed at the age of five in brother and four in sister. Both of them developed nephrotic syndrome shortly after the discovery of proteinuria. The nephrotic syndrome was resistant to corticosteroid, immunosuprresive agents or the combination of these drugs . Percutaneous renal biopsy in them revealed morphological and immunohistological features compatible to focal glomerular sclerosis. HLA typing in HLA-A, B, C and DR loci was identical to both. This observation suggests that genetic factors is associated with the pathogenesis of focal glomerular sclerosis.
