A 22-month-old girl with nephrotic syndrome and microcephaly is descri
bed. She had dismorphic facies and psychomotor retardation. Her parent
s were first-degree relatives and one of her siblings had died with ne
phrotic syndrome and renal failure in infancy. An autosomal recessive
inheritance is suggested. The diagnosis of this rare combination is di
scussed and the relevent literature is reviewed.