In our study, KF was found in only one woman (A3/32321455*bi) at the age of 33 with compound novel heterozygous missense variants c.4702C>T (p.Pro1568Ser) and c.3247G>C (p.Gly1083Arg) in COL4A3. Her biopsy demonstrated FSGS and a thinned and lamellated glomerulus basement membrane. The gene discussed is COL4A3; the disease is focal segmental glomerulosclerosis.