The case of a young man aged 18 years, attending to the Outpatient Service, Clinical and Surgical Teaching Hospital, Santa Clara, because of pathologic fracture in the anterior region of the jaw and history of hospitalization by pain and inflammation of mandibular gingiva with mobility of anterior teeth causing multiple extractions. The physical examination showed facial asymmetry by loss of normal chin configuration. Within investigations performed we had to rest on clinics, radiographical studies and anatomopathologic results. We think that the publication of this paper helps to enrich the scanty literature on this rare disease, which at international level is the tenth case involving the jaw and first in our country.