In a fetal autopsy series, we have explored the occurrence of renal tu
bular dysgenesis in twins. Renal tubular dysgenesis was found exclusiv
ely among those monozygotic twins with evidence of twin transfusion sy
ndrome, particularly in those donor twins with oligohydramnios and gro
wth restriction. We infer that hypotension in the donor twin of the tw
in transfusion syndrome pair is responsible for the failure of proxima
l convoluted tubule differentiation, and the disturbance of renal func
tion is manifested as oligohydramnios prenatally, and either oliguria
or tubular dysfunction postnatally.