BACKGROUND: Ossifying fibroma (OF) is a non-common benign fibrous-osseous lesion with highly aggressive behavior and tends to recur. Here, we report a case where Ossifying Fibroma (OF) was diagnosed in an adolescent female patient and was treated by marginal mandibular resection to avoid esthetic and functional defects in the future. CASE PRESENTATION: OF was diagnosed in a 13-year-old woman incidentally. Cone-Beam Computed Tomography (CBCT) examination showed a hypodense lesion compromising the vestibular table and 34 and 35 teeth. An incisional biopsy was performed to determine the histopathological diagnosis. After a multidisciplinary consensus, tooth extraction and surgical resection of the lesion were done. In the same way, bone reconstruction using the Khoury technique with an autologous graft of an external oblique line and with a mixture of autogenous and allogenic bone and fixation screws. Clinical and imaging follow-ups after 1, 4, 15, and 23 months were included, evidencing integration of the grafts in the treated zone. CONCLUSIONS: OF early identification and treatment are essential to minimize risks of losing tissues in young people. Also, to avoid negative consequences in the people´s quality of life associated with bone and teeth loss. This highlights the importance of radiographic surveillance, analyses, and interdisciplinary collaboration to optimize clinical outcomes in patients with similar conditions.