A slowly growing malignant tumor of the nail unit in a 74-year-old fem
ale is reported. At light microscopy, the tumor was composed of small
keratinous cysts with abrupt central keratinization, and of solid nest
s and strands of atypical keratinocytes filling the dermis and penetra
ting the phalangeal bone. In our view, this can be regarded as the mal
ignant analog of the keratin cysts arising from the nail bed epitheliu
m, as first reported by Samman in 1959. Since, according to several au
thors, the nail bed epithelium is comparable to the outer root sheath
of the follicle and since the reported tumor showed some analogies wit
h malignant proliferative trichilemmal cyst, it is suggested that this
new entity be designated as malignant proliferating onycholemmal cyst
.