During the last century of modern dermatology, more than 30 skin diseases have been described first by physicians from Japan. Many of those conditions were disorders of pigmentation and keratinization, which are quite common in Oriental patients. Since the late 1940s, a number of skin diseases first reported in Japan have gained attention internationally among them being Kimura disease, hypomelanosis of Ito, Kawasaki disease, adult T-cell leukemia/ lymphoma, eosinophilic pustular folliculitis, prurigo pigmentosa, and Ofuji's papuloerythroderma. In this article, we review skin diseases that were first established as distinct entities in Japan, in order to familiarize readers of the Western literature with these conditions.