Purpose: The aim of this study is to present a clinical case of a maxillary glandular odontogenic cyst (GOC), a rare development cyst that can have aggressive and recidivating behavior, emphasizing the treatment and preservation of this pathology. Case description: 36 years old brunette skin, female patient, was referred by the orthodontist due to present a volume increase in jaw with bulging and crepitation. Radiographic exams and aspiration were performed. After noticing the lesion contents, an incisional biopsy was performed and decompression was started with the placement of a drive tube. Since GOC diagnosis was confirmed, the patient underwent surgery to remove maxillary cyst by surgical enucleation, curettage and peripheral osteotomy. Conclusion: GOC is an uncommon pathology, recently recognized, with little known biological behavior. Therefore, it is suggested to carry out further investigations on the criteria for classification of variables commonly used in epidemiological studies in attempt to standardize and facilitate their comparisonto assist in a successful diagnosis.