Central giant cell lesion (CGCL) is an uncommon alteration of obscure etiology that, although benign, can be highly destructive. This paper presents the case of a patient with CGCL addressing its diagnosis and treatment. An 18-year-old male patient with facial asymmetry due to signifi cant volumetric increase in the right parotidmasseteric and submandibular regions with 9 months of evolution attended the Department of Dentistry of Mato Grosso Cancer Hospital. After clinical and imaginological examinations, an incisional biopsy was performed leading to the diagnostic of CGCL. With the negative results for hyperparathyroidism, the lesion reduction was attempted with corticosteroids infi ltrations. Due to the lesion extension and the lack of response to the infi ltrations, the resection was performed. The patient is in attendance for 2 years without any symptoms of recurrence. Although more prevalent among female, young adults and elderly, the CGCL may present in other patients and the professional must be aware. The therapy of choice in this pathology varies with the aggressiveness and extension of the lesion. More aggressive lesions can lead to more extreme clinical management and involve the need for surgical resection.