Odontogenic keratocyst (OKC) characterized by a single lesion, with greater frequency in the mandibular jaw. The diagnostic approach is based on a combined analysis of the medical history, the clinical appearance and the radiographic appearance. The diagnosis may be confirmed by the anatomical pathology report. Finally, treatment consists of surgical excision and follow up is characterized by a high rate of recurrence. The authors report an unusal case of OKC of the upper jaw and review the various diagnoses and therapeutics.