A 49-year-old Japanese man with typical features of nevoid basal cell epithelioma syndrome is reported. The patient had cystic lesions of the jaw bone, multiple basal cell epitheliomas of the scalp and face, pits on the palms and soles, bifid ribs, scoliosis and calcification of the falx cerebri. In Japan five certain cases have been reported previously. We added one new case and reviewed the other five cases briefly.