The odontogenic keratocyst is the third most common cyst of the jaws, after the follicular and radicular cyst. Keratocysts most commonly occur as single lesions in the jaw of otherwise healthy persons. Multiple odontogenic keratocysts are a well-recognized feature of the nevoid basal cell carcinoma syndrome. The mandible, especially the third molar region, the angle of the mandible and the ascending ramus are involved far more frequently than the maxilla. Clinically, the cysts often remain asymptomatic and there are two specific histological entities: the orthokeratinized and the parakeratinized odontogenic keratocyst. Different surgical treatment options like marsupialization, enucleation with curettage or peripheral ostectomy, and osseous resection (marginal or segmental) have been discussed in the literature with variable rates of recurrence. Besides a predilection for recurrence, the keratocysts, in contrast to other odontogenic cysts, show a more aggressive clinical behavior and demonstrate a high mitotic count and higher turnover rate of the epithelium. This led to the tentative suggestion that the keratocyst might be a benign cystic neoplasm rather than simply an odontogenic cyst.

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