A new case of Aase-Smith syndrome has ben reported with additional fin
dings, including microcephaly, axial rotation of the kidneys, preaxial
polydactyly and leukopenia. The, patient had almost complete clinical
remission with corticosteroid treatment. The findings in this case su
ggest that it is very difficult to differentiate Aase-Smith syndrome f
rom Blackfan-Diamond and Fanconi's anemias as well as from those cases
reported by Murphy and Lubin, and Jones and Thompson.