The odontogenic keratocyst is a lesion with specific clinical and histopathological aspects. The World Health Organization (WHO) in 2017 reclassified it from a tumor to a cyst. It is characterized as a cyst of epithelial development of the jaws, arising from the remains of the dental blade. It represents 3 % to 11 % of all odontogenic cysts and 7 to 11 % of cysts of the gnatic bones. It stands out for its aggressive nature and high relapsing potential. Most of the cases are diagnosed in individuals between 10 and 40 years old, with a mild preference for the masculine gender, occurring more in the mandible. Radiographically, it is radiolucent and well delimited, predominantly unilocular, and may cause displacement of adjacent teeth. The present study aims to report a clinical case of a female 25 years old patient, presenting an intra-osseous lesion in the maxilla (posterior, left side), asymptomatic, with a slight increase in intraoral buccal volume, containing the tooth 28, with a diagnostic hypothesis of Odontogenic Keratocyst. The patient was submitted to the surgical decompression treatment, with cystic fluid puncture, biopsy of the lesion and posterior anatomopathological examination. The enucleation of the tumor was performed using LeFort I osteotomy of maxilla and reconstruction with titanium mesh. There is radiographic evidence of bone repair and lesion reduction. The patient is in periodic follow-up of 4 years and does not present clinical and radiographic signs of relapse. Due to the aggressiveness of the odontogenic keratocyst, the relapse rate is high. The knowledge of the techniques recommended for the treatment of Odontogenic Keratocysts and the clinical and radiographic follow-up of the patient demonstrate a gradual decrease of the lumen of the lesion and suggest local bone neoformation, favoring the prognosis of the case.