Two siblings with primary nephrotic syndrome are presented. The parents of the patients were first cousins. There was no reported history of renal disease in three generations of their family. The nephrotic syndrome developed at the age of one year in both siblings. They were both resistant to immunosuppressive treatment. The older child died of septicemia six months following the clinical appearance of the nephrotic syndrome. A renal biopsy, which was performed only on the younger child, showed typical findings of focal glomerulosclerosis.

Keywords: nephrotic syndrome (NS), focal segmental glomerulosclerosis (FSGS)