Basal cell nevus syndrome (BCNS) is a hereditary condition tansmitted as an autosomal dominant trait that exhibits high penetrance and variable expressivity. There are many potential complications which have been reported in association with the naevoid basal cell carcinoma syndrome. The data upon which this review is based has been retrieved from published case reports in Korean dental and medical literatures between the years 1981 to 2002. This survey included unpublished 3 patients by reviewing patients files and charts from the Dental Hospital, Chosun University during same periods. We found 33 subjects who met the diagnostic criteria for BCNS reported by Evans et al. Relative frequencies of associated complications are presented and compared with those of the recent English survey. The frequencies of most manifestations are similar. Odontogenic keratocyst (OKC) and palmar and/or plantar pits, and hypertelorism were the most frequently observed anomalies. OKCs are often the first signs of BCNS and can be detected in patients younger than 9 years of age. However, the relatively low frequency of basal cell carcinomas and ectopic calcification were two major difference. This study more clearly defines the possible complications of the syndrome and gives earlier guidelines for counselling and screening affected and at risk persons.