Until recently the newborn infant with a large head and short limbs who died soon after birth was assumed to have achondroplasia. This assumption has been shown to be incorrect by the delineation of at least two additional disorders, thanatophoric dwarfism1 and achondrogenesis,2 which also cause this physical appearance. Of the two, achondrogenesis has more definite genetic significance to the family in that it is an autosomal recessive disorder.3 Recent reviews4,5 indicate that at least 22 infants with achondrogenesis have been reported. Because of the importance of proper recognition of this disorder by pediatricians, this additional case of achondrogenesis is being reported.