The keratocystic odontogenic tumor is a benign odontogenic cystic neoplasia characterized by its thin, squamous epithelium with superficial parakeratosis. It has the potential for infiltration and local aggressiveness and has a high rate of recurrence. This neoplasia is predominantly found in males and people of white origin. The mandible is the most frequently involved site, in particular the third molar region, mandibular angle, and ramus. It has a mandible-maxilla ratio of 2:1. Only about twenty cases of peripheral keratocystic odontogenic tumors (PKCOT) have been reported in the international literature. This study presents a case of PKCOT localized in the anterior region of the maxilla, on the vestibular side of the upper left lateral incisor and the upper left canine. The diagnosis and treatment procedures, as based on the literature, are also discussed. Key words:Odontogenic cysts, odontogenic tumors, keratocyst, keratocystic odontogenic tumor.