Background: Despite the common occurrence of localized gingival enlargements, which often represent reactive lesions, the temporal and spatial association of such a lesion with a central jaw lesion has not been reported. The purpose of this case report is to present the exceptional combination of a peripheral ossifying fibroma and a central odontogenic fibroma. The differential diagnosis and management of each lesion is reviewed. Methods: A 45-year-old black female presented with a chief complaint of a painless protuberance in the left mandible of 1-year duration. Clinical and radiographic examination revealed a gingival enlargement localized between teeth #21 and #23 and a multilocular radiolucent lesion with radiopaque foci in the same area. Excisional biopsy of the gingival lesion and incisional biopsy of the central lesion were performed, and specimens were submitted for histopathological analysis. Results: Biopsy of the gingival lesion revealed stratifled squamous epithelium and highly cellular fibroblastic component presenting central areas of calcification, features consistent with a diagnosis of peripheral ossifying fibroma. The central lesion was characterized by cellular fibrous tissue admixed with rests of odonlogenic epithelium and few calcification areas, features consistent with a diagnosis of central odontogenic fibroma/World Health Organization type. Subsequently, the central lesion was enucleated. After 1-year follow-up, no recurrence has been observed. Conclusions: The combination of a rare central lesion with a common gingival lesion may present unique diagnostic and therapeutic challenges. Clinician awareness regarding the possibility of such a combined presentation and its implications will help to ensure optimal treatment outcomes.78713541359