This report describes the following developmental defects in two siblings: shortened extremities, coarse facial features, abnormally thickned palms and soles with an abnormal dermatoglyphic pattern, lack of cartilage formation of both ears, cleft of the soft palate, marked hydronephrosis due to ureteral stenosis, huge inguinal hernias in the male, and a bicornuate uterus in the female. Both children failed to develop motor control and died at 4 and 5 months, respectively. It is suggested that they represent a previously unidentified syndrome of congenital defects, possibly due to an autosomal recessive gene.