Objective The purpose of this study was to evaluate the clinical manifestation, diagnosis and treatment of basal cell naevus syndrome（BCNS）. Methods Reviewed the procedure of diagnosis and treatment by analyzing the records of 4 inpatients of BCNS in General Hospital Affiliated to Tianjin Medical University from 1999 to 2007. Results The inpatients characterized with typical facial features, cutaneous basal cell naevus（carcinoma） were obviously seen on the skin, palmar or plantar pits were viewed, bilamellar calcification of falx cerebri, bifid, fused or markedly splayed ribs were found, and histologically confirmed odontogenic keratocysts of jaw could be detected in most cases of BCNS. No family history was found in all cases of the group. Conclusion
Multiple odontogenic keratocysts of jaw is one of the most important clinical manifestation of BCNS. The correct and prompt treatment will provide the patients with better life quality.
