A case is reported of basal cell nevus syndrome in a 9-year-old girl, presentingmultiple jaw cysts, broad nasal base, ocular hyperterolism, incomplete osseous bridging of sella turcica and abnormal ribs. From the family investigations, it turned out that the syndrome occurred also in her mother and the mother's brother. The syndrome of these cases was familial, but chromosome aberration was not confirmed. In these three cases, common symptoms were multiple jaw cysts and abnormal rib. Such familial cases are considered rare in Japan.
