Two newborns with glomerulocystic kidney disease manifesting as late onset
oligohydramnion and neonatal anuria, yet without severe respiratory distres
s, are presented. They had a similar perinatal course and associated clinic
al manifestations. No associated congenital or inherited malformation syndr
ome could be defined. Both infants' parents were first degree cousins and b
elonged to the same small Bedouin tribe, and neither they nor the infants'
siblings had polycystic kidneys or renal insufficiency, pointing to either
a possible genetic etiology or a common external toxic exposure.