The odontogenic keratocyst (OKC) is a clinic and histologically distinct form of odontogenic cyst. It is known for its aggressive behaviour, high recurrence rate and may be in association with nevoid basal cell carcinoma syndrome. Recently was reported that the lost of human patched (ptch), a tumour suppressor gene, is the possible molecular origin of the OKC. This report reviews the clinic, histologic and molecular characteristics of OKCs. Treatment and recurrence are discussed too.