Four cases of an until now undescribed syndrome have been observed in
Berne in the last 40 years. All four cases are members of the same fam
ily and have occurred in three consecutive generations. They present w
ith a U-shaped palatal cleft, microstomia, hypoplasia of the mandibula
and a partial anodontia. An autosomal dominant heredity was demonstra
ted. Karyograms have been made in three of the patients and in all pat
ients showed an anomaly in the form of a ''fragile site'' in one chrom
osome (16 fra 16 [q22]). Surgical and orthopedic treatments were diffi
cult.