A family with dominant inheritance of a rare renal malformation is reported
. The father and one son had left crossed fused ectopic and dysplastic kidn
eys and another son had a horseshoe kidney and vesicoureteral reflux. We di
scuss various potential pathogenetic mechanisms and propose that a defect i
n the timing of the proper reciprocal induction of the ureteric bud and the
metanephric blastema is involved.