Basal cell nevus syndrome is a hereditary disease of an autosomal dominant trait with variable conditions such as basal cell carcinomas of the skin, deformity of rib, fusion of vertebrae, mental retardation, hypertelorism, and multiple odontogenic keratocysts. A 32 years old man with pus discharge from fistula on the vestibule of left upper 1st molar visited to Chosun University Dental Hospital. Radiographic evaluation revealed multiple maxillary and mandibular cysts that had multilocular radiolucency on left mandibular body area, thining of inferior border of left border of ramus and well defined unilocular radiolucency above right upper 1st and 2nd molar and from left upper 1st premolar to 2nd molar. In chest PA view, he had a forked rib in the left 4th rib and in skull PA view the calcification of falx cerebri was observed. There was not any skin lesion. After the preliminary evaluation, the patient was diagnosed with basal cell nevus syndrome and he underwent marsupialization for decreasing the size of cystic lesion and came to hospital for dressing 3days a week. As time goes by, the size of lesion decreased. So, one and half year after marsupialization, he underwent cyst enucleation and iliac bone graft for the mandibular lesion and buccal fat pad grafts for the maxillary lesions. After the surgery, the patient experienced normal healing without any complications and he is on long-term follow-up.