We present premature female twin fetuses with concordant extremely sho
rtened ribs, shea Limbs, macrocephaly, median cleft upper lip and faci
al dysmorphism. Based on radiological criteria and the pattern of asso
ciated abnormalities, a lethal short rib-polydactyly syndrome (Beemer-
Langer type) was diagnosed. The differential diagnosis of this entity
is discussed.