We present a family affected with distal arthrogryposis type IIB (DA IIB) i
n three generations. The coincidental finding of Dandy-Walker anomaly in th
e proposita arouses the question whether Dandy-Walker anomaly is part of th
e DA IIB spectrum or whether the Aase-Smith syndrome belongs to the DA spec
trum. (C) 2000 Wiley-Liss, Inc.