抄録
Thanatophoric dwarfism is one of lethal neonatal shortlimbed dwarfism. A case of Thanatophoric dwarfism (32 weeks male fetus) is repoted. This case indicates the charactristic radiograohic features of a large skull (clover leaf skull), narrow thorax, short ribs, platyspondyly, increased intervertebral spaces and extremely short bowed tubular bones particularly in the lower extremities (like telephone receiver). The temporal bone histopathologic findings are sever endolymphatic collaps, roundshaped stapes, deepened internal auditory canal endolymphatic sac surrounded with dick connective tissue.